NCIt Code NCIt PT Subset PT Subset SY NCIt Definition Subset Definition NCIt Code of First Parent First Parent NCIt Code of Second Parent Second Parent NCIt Code of Subset PT of NICHD Subset Subset Antiquated AQ Subset Antiquated AQS C247 ACE Inhibitor ACE Inhibitor Angiotensin-Converting Enzyme Inhibitor Any substance that inhibits angiotensin-converting enzyme (ACE), an enzyme that catalyzes the conversion of angiotensin I to angiotensin II. Inhibition of ACE results in a reduction in angiotensin II and angiotensin II-induced aldosterone secretion, causing vasodilation and natriuresis. A pharmaceutical agent that inhibits angiotensin-converting enzyme (ACE), which catalyzes the conversion of angiotensin I to angiotensin II. Inhibition of ACE results in a reduction in angiotensin II and angiotensin II-induced aldosterone secretion, causing vasodilation and natriuresis. It is primarily used for the reduction of proteinuria and the treatment of hypertension. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C119989 Acute Anterior Uveitis Acute Anterior Uveitis Sudden onset of anterior uveitis, usually unilateral, and associated with pain, erythema, photophobia, and blurred vision. Often associated with HLA-B27, with or without co-existing spondyloarthritis. Sudden onset of anterior uveitis, usually unilateral, and associated with pain, erythema, photophobia, and blurred vision. Often associated with HLA-B27, with or without co-existing spondyloarthritis. C35109 Anterior Uveitis C118468 Pediatric Rheumatology Terminology C121340 American College of Rheumatology Classification Criteria for Juvenile Rheumatoid Arthritis American College of Rheumatology Classification Criteria for Juvenile Rheumatoid Arthritis ACR Classification Criteria for JRA A set of antiquated criteria used to assign children with chronic arthritis to one of the three mutually exclusive categories. A set of antiquated criteria used to assign children with chronic arthritis to one of the three mutually exclusive categories. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121336 American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus ACR Classification Criteria for SLE The classification of systemic lupus erythematosus based on eleven criteria. Intended to be used as inclusion criteria for clinical studies, a person is defined as having systemic lupus erythematosus if any four or more of the eleven criteria are present, serially or simultaneously, during any interval of observation. The classification of systemic lupus erythematosus based on eleven criteria. Intended to be used as inclusion criteria for clinical studies, a person is defined as having systemic lupus erythematosus if any four or more of the eleven criteria are present, serially or simultaneously, during any interval of observation. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121341 American College of Rheumatology Pediatric Core Set for Juvenile Arthritis American College of Rheumatology Pediatric Core Set for Juvenile Arthritis ACR Pediatric Core Set for Juvenile Arthritis A set of criteria used to assess clinical response (improvement) or worsening (flare), in juvenile idiopathic arthritis clinical trials. A set of criteria used to assess clinical response (improvement) or worsening (flare), in juvenile idiopathic arthritis clinical trials. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C66930 Angiotensin II Receptor Antagonist Angiotensin II Receptor Antagonist ARB A class of agents that act by selectively inhibiting angiotensin II receptor activation in the renin-angiotensin-aldosterone system. Angiotensin II receptor antagonists bind to and block the activation of angiotensin II type 1 (AT1) receptors, thereby reducing production and secretion of aldosterone, amongst other actions. The combined effects result in reduction of blood pressure. Although this class of agents is also used in diabetic nephropathy (kidney damage due to diabetes) and congestive heart failure, it is primarily used for the treatment of hypertension in cases where the patient is intolerant of ACE inhibitor therapy. A pharmaceutical agent that selectively inhibits angiotensin II receptor activation in the renin-angiotensin-aldosterone system reducing production and secretion of aldosterone, amongst other actions. The combined effects result in reduction of blood pressure. It is primarily used for the reduction of proteinuria and the treatment of hypertension in cases where the patient is intolerant of ACE inhibitor therapy. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C84564 Ankylosing Spondylitis Ankylosing Spondylitis An autoimmune chronic inflammatory disorder characterized by inflammation in the vertebral joints of the spine and sacroiliac joints. It predominantly affects young males. Patients present with stiffness and pain in the spine. An immune-mediated disease characterized by inflammation in the axial skeleton including sacroiliac joints, vertebrae and vertebral joints. Peripheral joints may be involved as well. Clinical features can include low back pain and stiffness, limited motion of the lumbar spine, and limited chest expansion. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C35109 Anterior Uveitis Anterior Uveitis Inflammation of the iris and anterior chamber of the eye. Inflammation of the anterior uveal tract. C26909 Uveitis C118468 Pediatric Rheumatology Terminology C121328 Anti-Beta-2 Glycoprotein 1 Antibody Anti-Beta-2 Glycoprotein 1 Antibody AAHA|AB2GP|Anti-Apolipoprotein H Antibody|Anti-Apolipoprotein H|Anti-B2GP1|Anti-Beta-2 Glycoprotein I Antibody An antiphospholipid autoantibody that reacts with beta-2-glycoprotein 1, and a member of family of autoantibodies that cause antiphospholipid antibody syndrome and may be found in system lupus erythematosus. An antiphospholipid autoantibody that reacts with beta-2-glycoprotein 1, and a member of family of autoantibodies that cause antiphospholipid antibody syndrome and may be found in system lupus erythematosus. C16295 Antibody C118468 Pediatric Rheumatology Terminology C114565 Anti-ds DNA Antibody Anti-ds DNA Antibody Anti-Double Stranded DNA Antibody|Anti-dsDNA Antibody A type of antinuclear antibody that specifically targets double stranded DNA; while these antibodies are sometimes detectable in multiple different autoimmune disorders, their presence is generally considered highly specific for systemic lupus erythematosus. An antinuclear antibody that specifically recognizes double-stranded DNA. Their presence is highly specific for systemic lupus erythematosus. C16295 Antibody C118468 Pediatric Rheumatology Terminology C121323 Anti-La Antibody Anti-La Antibody Anti-SSB/La Antibody An antinuclear antibody directed against a 47 kDa RNA-binding protein in the nucleus. They can be associated with Sjogren syndrome and system lupus erythematosus. Maternal anti-La antibody positivity can be associated with neonatal lupus. An antinuclear antibody directed against a 47 kDa RNA-binding protein in the nucleus. They can be associated with Sjogren syndrome and system lupus erythematosus. Maternal anti-La antibody positivity can be associated with neonatal lupus. C16295 Antibody C118468 Pediatric Rheumatology Terminology C271 Antimalarial Agent Anti-Malarial Agent Anti-Malarial Drug Agents used to treat malaria, and are usually classified based on action against plasmodia at different stages in their life cycle in the human. A pharmaceutical agent that cures or prevents malaria. Certain anti-malarials, specifically chloroquine derivatives, have been used in the treatment of autoimmune disease. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C121325 Anti-Ribonucleoprotein Antibody Anti-Ribonucleoprotein Antibody Anti-RNP Antibody An antinuclear antibody directed against U1 snRNP, that is strongly associated with mixed connective tissue disease and commonly detected in lupus. An antinuclear antibody directed against U1 snRNP, that is strongly associated with mixed connective tissue disease and commonly detected in lupus. C16295 Antibody C118468 Pediatric Rheumatology Terminology C121322 Anti-Ro Antibody Anti-Ro Antibody Anti-SSA/Ro Antibody An antinuclear antibody directed against 52 or 60 kDa RNA-binding proteins in the nucleus. They can be associated with multiple autoimmune disorders, primarily Sjogren syndrome and system lupus erythematosus. Maternal anti-Ro antibody positivity can be associated with neonatal lupus. An antinuclear antibody directed against 52 or 60 kDa RNA-binding proteins in the nucleus. They can be associated with multiple autoimmune disorders, primarily Sjogren syndrome and system lupus erythematosus. Maternal anti-Ro antibody positivity can be associated with neonatal lupus. C16295 Antibody C118468 Pediatric Rheumatology Terminology C121324 Anti-Smith Antibody Anti-Smith Antibody Anti-Sm Antibody An antinuclear antibody directed against small nuclear ribonucleoproteins (snRNPs), that is highly specific, but has poor sensitivity for systemic lupus erythematosus (SLE). Presence of anti-Sm antibodies are associated with central nervous system, kidney, lung and cardiac involvement in SLE, but are not indicative of disease activity. An antinuclear antibody directed against small nuclear ribonucleoproteins (snRNPs), that is highly specific, but has poor sensitivity for systemic lupus erythematosus (SLE). Presence of anti-Sm antibodies are associated with central nervous system, kidney, lung and cardiac involvement in SLE, but are not indicative of disease activity. C16295 Antibody C118468 Pediatric Rheumatology Terminology C16295 Antibody Antibody A type of protein made by B lymphocytes in response to a foreign substance (antigen). Each antibody only binds to a specific antigen, helping to destroy the antigen directly or by assisting white blood cells to destroy the antigen. C13236 Body Fluid or Substance C118468 Pediatric Rheumatology Terminology C95408 Anticardiolipin Antibody Anticardiolipin Antibody ACA|aCL An autoantibody that reacts with cardiolipin. Different immunoglobulin subclasses and isotypes are associated with different diseases and conditions. Some anticardiolipin antibodies prolong phospholipid dependent clotting while others are associated with spontaneous abortion. An antiphospholipid autoantibody that reacts with cardiolipin. It can be associated with antiphospholipid antibody syndrome and system lupus erythematosus. C16295 Antibody C118468 Pediatric Rheumatology Terminology C70547 Antinuclear Antibody Antinuclear Antibody ANA An autoimmune antibody that is directed against structures within the nucleus of the cell. A class of autoantibodies that are directed against structures within the nucleus of the cell. They can be associated with rheumatic disease, but may be present in unaffected individuals. C16295 Antibody C118468 Pediatric Rheumatology Terminology C121327 Antiphospholipid Antibody Antiphospholipid Antibody APLA A class of autoantibodies directed against phospholipids or phospholipoproteins. Its presence may indicate an increased risk for hypercoagulability, pregnancy loss or autoimmune disease. A class of autoantibodies directed against phospholipids or phospholipoproteins. Its presence may indicate an increased risk for hypercoagulability, pregnancy loss or autoimmune disease. C16295 Antibody C118468 Pediatric Rheumatology Terminology C61283 Antiphospholipid Syndrome Antiphospholipid Antibody Syndrome Antiphospholipid Syndrome A disorder caused by the presence of autoantibodies directed against phospholipids, causing a hypercoaguable state, which may result in blood clots, stroke, heart attack, and in women, significant pregnancy-related complications, including miscarriage and still birth. The syndrome is often associated with other autoimmune disorders, most commonly lupus erythematosus, and infections, including syphilis and Lyme disease. A disorder caused by the presence of autoantibodies directed against phospholipids, causing a hypercoaguable state, which may result in blood clots, stroke, heart attack, and in women, significant pregnancy-related complications, including miscarriage and stillbirth. The syndrome is often associated with other autoimmune disorders, most commonly lupus erythematosus, and certain infections. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C15191 Pheresis Apheresis Hemapheresis|Pheresis A procedure that collects a component of the peripheral blood while returning the rest to the donor. A procedure that collects a component of the peripheral blood while returning the rest to the donor's circulation. C49236 Therapeutic Procedure C118468 Pediatric Rheumatology Terminology C62546 Canker Sore Aphthous Ulcer Aphthous Stomatitis|Canker Sore A recurrent disease of the oral mucosa of unknown etiology. It is characterized by small white ulcerative lesions, single or multiple, round or oval, lasting for 7-14 days and healing without scarring. A term used to describe oral, non-keratinized mucosal ulcerations that are shallow, round to oval with a grayish base, and are usually painful. Recurrent aphthous ulcers are often associated with predisposing factors such as stress, familial tendency, trauma, drug hypersensitivity, or underlying disease such as inflammatory bowel disease, systemic lupus erythematosus, and Behcet disease. C3426 Ulcer C118468 Pediatric Rheumatology Terminology C2881 Arrhythmia Arrhythmia Cardiac Arrhythmia Any variation from the normal rate or rhythm (which may include the origin of the impulse and/or its subsequent propagation) in the heart. Any variation from the normal rate or rhythm (which may include the origin of the impulse and/or its subsequent propagation) in the heart. C78245 Cardiac Conduction Disorder C118468 Pediatric Rheumatology Terminology C34399 Arteritis Arteritis An inflammatory process affecting an artery. Inflammation of an artery. C35117 Vascular Disorder C118468 Pediatric Rheumatology Terminology C50464 Arthralgia Arthralgia Joint Pain Pain in a joint. Pain in a joint. C3303 Pain C100104 Sign or Symptom C118468 Pediatric Rheumatology Terminology C2883 Arthritis Arthritis An inflammatory process affecting a joint. Causes include infection, autoimmune processes, degenerative processes, and trauma. Signs and symptoms may include swelling around the affected joint and pain. Inflammation of the joint tissues of any etiology. Signs and symptoms may include pain, swelling, warmth, guarding, limited range of motion or overlying erythema. C107377 Musculoskeletal System Disorder C118468 Pediatric Rheumatology Terminology C2889 Autoimmune Disease Autoimmune Disease Autoimmune Disorder A disorder resulting from loss of function or tissue destruction of an organ or multiple organs, arising from humoral or cellular immune responses of the individual to his own tissue constituents. It may be systemic (e.g., systemic lupus erythematosus), or organ specific, (e.g., thyroiditis). A disorder that may result in abnormality, impaired function or damage of one or more organs or tissues, arising from autoreactive humoral or cellular immune responses. C3507 Immune System Disorder C118468 Pediatric Rheumatology Terminology C119050 Autoinflammatory Syndrome Autoinflammatory Syndrome A group of disorders of the innate immune system characterized by attacks of seemingly unprovoked inflammation without significant levels of either autoantibodies or autoreactive T cells more characteristic of autoimmune disease. A group of disorders of the innate immune system characterized by attacks of seemingly unprovoked inflammation without significant levels of either autoantibodies or autoreactive T cells more characteristic of autoimmune disease. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C121355 Bath Ankylosing Spondylitis Disease Activity Index Bath Ankylosing Spondylitis Disease Activity Index BASDAI A clinical index used to assess the activity of ankylosing spondylitis that has been validated in adults. A clinical index used to assess the activity of ankylosing spondylitis that has been validated in adults. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C34416 Behcet Syndrome Behcet Syndrome Behcet Disease|Behcet's Syndrome|Behçet Disease|Behçet's Syndrome A rare chronic inflammatory disorder of unknown etiology. It is characterized by the development of ulcers in the mouth and genital region and uveitis. Other signs and symptoms include arthritis, deep vein thrombosis and superficial thrombophlebitis. A systemic vasculitis affecting both arteries and veins which manifests primarily as recurrent oral and genital ulceration, uveitis, characteristic rash, and arthritis. It may also cause central nervous system disease, gastrointestinal inflammation or ulceration, or thrombophlebitis. C26912 Vasculitis C118468 Pediatric Rheumatology Terminology Behçet-Adamantiades Syndrome|Morbus Behçet's Syndrome|Silk Road Disease C307 Biological Agent Biologic Biological Agent|Biological Drug|Biologicals|Biopharmaceuticals Biotechnology-derived pharmaceutical agents made from living organisms or their products. Includes recombinant proteins, monoclonal antibodies, and nucleic acids. A class of pharmaceutical agents made from living organisms or their products that includes recombinant proteins, antibodies, and nucleic acids. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C121334 Biologic Disease-Modifying Antirheumatic Drug Biologic Disease-Modifying Antirheumatic Drug Biologic DMARD A biologic used to alter the progression of autoimmune disease. A biologic used to alter the progression of autoimmune disease. C121332 Disease-Modifying Antirheumatic Drug C118468 Pediatric Rheumatology Terminology C121343 Birmingham Vasculitis Activity Score Birmingham Vasculitis Activity Score BVAS A set of criteria used to determine the activity of vasculitis. A set of criteria used to determine the activity of vasculitis. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C116794 Blau Syndrome Blau Syndrome Pediatric Granulomatous Arthritis An autoinflammatory disease caused by a NOD2 gene mutation, usually presenting in children younger than age four, and characterized by granulomatous dermatitis, arthritis with synovitis, and uveitis. An autoinflammatory disease caused by a NOD2 gene mutation, usually presenting in children younger than age four, and characterized by granulomatous dermatitis, arthritis with synovitis, and uveitis. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C121352 British Isles Lupus Assessment Group Index British Isles Lupus Assessment Group Index BILAG Index An organ-specific assessment intended as a clinical decision tool. It evaluates specific manifestations over the previous four weeks in a total of eight organs systems, nine in the revised Index: constitutional, mucocutaneous, neuropsychiatric, musculoskeletal, cardiorespiratory, gastrointestinal, ophthalmic, renal, and hematological. Activity in each organ system is scored from most active disease to no previous activity. An organ-specific assessment intended as a clinical decision tool. It evaluates specific manifestations over the previous four weeks in a total of eight organs systems, nine in the revised Index: constitutional, mucocutaneous, neuropsychiatric, musculoskeletal, cardiorespiratory, gastrointestinal, ophthalmic, renal, and hematological. Activity in each organ system is scored from most active disease to no previous activity. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C117104 Bullous Systemic Lupus Erythematosus Bullous Systemic Lupus Erythematosus BSLE A manifestation of systemic lupus erythematosus with a widespread vesiculobullous eruption. A manifestation of systemic lupus erythematosus with a widespread vesiculobullous eruption. C3201 Systemic Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C60651 C-Reactive Protein C-Reactive Protein CRP C-reactive protein (224 aa, ~25 kDa) is encoded by the human CRP gene. This protein is cleaved during biological activation and is associated with host defense mechanisms and inflammatory responses. An acute phase reactant used in the evaluation of systemic inflammation. C13236 Body Fluid or Substance C118468 Pediatric Rheumatology Terminology C119990 C1q Deficiency C1q Deficiency Lack of production of functional C1q proteins, due to a genetic defect. Virtually 100% of patients with a C1q deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. Lack of production of functional C1q proteins, due to a genetic defect. Virtually 100% of patients with a C1q deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. C4691 Complement Deficiency C118468 Pediatric Rheumatology Terminology C119991 C1r/C1s Deficiency C1r/C1s Deficiency Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. C4691 Complement Deficiency C118468 Pediatric Rheumatology Terminology C119992 C2 Deficiency C2 Deficiency Lack of production of functional C2 protein, due to a genetic defect. It is the most common genetic complement deficiency. Ten percent of C2 deficient patient will develop systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. Lack of production of functional C2 protein, due to a genetic defect. It is the most common genetic complement deficiency. Ten percent of C2 deficient patient will develop a systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. C4691 Complement Deficiency C118468 Pediatric Rheumatology Terminology C9468 C3 Deficiency C3 Deficiency A rare genetic disorder with an autosomal recessive pattern of inheritance. It is caused by the ineffective or decreased biosynthesis of the third complement component, C3. C3 deficiency may also be acquired acutely post-infection or chronically from co-morbid autoimmune disorders. If C3 is adequately synthesized, its rapid depletion may result in a functional deficiency. Clinical signs of the inherited deficiency present within the first decade of life and are consistent with the signs of recurrent systemic infection or immune complex disease. Deficiency of serum C3 and its major cleavage product, C3b, will decrease the effective humoral immune response to encapsulated bacteria. Deficiency of C3 also impairs clearance of circulating immune complexes and therefore predisposes to rheumatic and renal disease. Lack of production of functional C3 protein, due to a genetic defect. Patients present at an early age with overwhelming infections with encapsulated bacteria. Patients may also present with systemic lupus erythematosus, autoimmune glomerulonephritis, or juvenile idiopathic arthritis-like syndromes. C4691 Complement Deficiency C118468 Pediatric Rheumatology Terminology C119993 C4 Deficiency C4 Deficiency Lack of production of functional C4 protein, due to a genetic defect requiring homozygous loss of both the C4A and C4B genetic paralogs. Approximately 75% of patients with a C4 deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. Lack of production of functional C4 protein, due to a genetic defect requiring homozygous loss of both the C4A and C4B genetic paralogs. Approximately 75% of patients with a C4 deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae. C4691 Complement Deficiency C118468 Pediatric Rheumatology Terminology C333 Calcium Channel Blocker Calcium Channel Blocker CCB A pharmaceutical agent that inhibits the movement of calcium across through calcium channels in the cell membrane, preventing or decreasing the amount of calcium able to enter the cell. These drugs are used for their chronotropic, antihypertensive and vasodilatory effects. A pharmaceutical agent that inhibits the movement of calcium across through calcium channels in the cell membrane, preventing or decreasing the amount of calcium able to enter the cell. These drugs are used for their chronotropic, antihypertensive and vasodilatory effects. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C116919 Central Nervous System Lupus Central Nervous System Lupus CNS Lupus Inflammation that includes the brain, spinal cord and surrounding tissues secondary to systemic lupus erythematosus (SLE); it is associated with neurological and/or psychiatric features. Inflammation that includes the brain, spinal cord and surrounding tissues secondary to systemic lupus erythematosus (SLE); it is associated with neurological and/or psychiatric features. C27153 Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C121330 Child Health Questionnaire Child Health Questionnaire CHQ A collection of general quality of life surveys that measures physical and psychosocial concepts in children. A collection of general quality of life surveys that measures physical and psychosocial concepts in children. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121329 Childhood Health Assessment Questionnaire Childhood Health Assessment Questionnaire C-HAQ|CHAQ A pediatric modification of the Stanford Health Assessment Questionnaire (HAQ) used in the evaluation of functional disability and discomfort in children. A pediatric modification of the Stanford Health Assessment Questionnaire (HAQ) used in the evaluation of functional disability and discomfort in children. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121344 Childhood Myositis Assessment Scale Childhood Myositis Assessment Scale CMAS An observational performance-based instrument developed to evaluate muscle strength, physical function, and endurance in children with juvenile idiopathic inflammatory myopathy. An observational performance-based instrument developed to evaluate muscle strength, physical function, and endurance in children with juvenile idiopathic inflammatory myopathy. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C116798 Childhood-Onset Systemic Lupus Erythematosus Childhood-Onset Systemic Lupus Erythematosus CSLE|JSLE|Juvenile SLE|PSLE|Pediatric SLE Systemic lupus erythematosus (SLE) diagnosed in individuals under the age of eighteen. Children frequently have multi-organ involvement and acute disease onset. Symptoms include fever, arthritis, skin lesions, anemia, and fatigue. Systemic Lupus Erythematosus (SLE) diagnosed in individuals under the age of 18. Children frequently have multi-organ involvement and acute disease onset. C3201 Systemic Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C121321 Chloroquine Compound Chloroquine Compound A pharmaceutical agent initially used to cure or prevent malaria. A principal rheumatologic indication is the treatment of autoimmune disease including dermatomyositis, lupus, rheumatoid arthritis, and Sjogren syndrome. A pharmaceutical agent initially used to cure or prevent malaria. A principal rheumatologic indication is the treatment of autoimmune disease including dermatomyositis, lupus, rheumatoid arthritis, and Sjogren syndrome. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C119042 Chronic Recurrent Multifocal Osteomyelitis Chronic Recurrent Multifocal Osteomyelitis CRMO|NBO|Non-bacterial Osteomyelitis An autoinflammatory disease characterized by sterile bone lesions which are multifocal and/or recurrent. An autoinflammatory disease characterized by sterile bone lesions which are multifocal and/or recurrent. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C34476 Chronic Iridocyclitis Chronic Uveitis Chronic Iridocyclitis Uveitis that is usually insidious in onset, bilateral, and can be asymptomatic, most often affecting the anterior uveal tract, including the iris and ciliary body, and can result in long-term complications. It is most commonly associated with certain forms of juvenile idiopathic arthritis, but may occur independent of arthritis. Uveitis that is usually insidious in onset, bilateral, and can be asymptomatic. Usually affects the anterior uveal tract including the iris and ciliary body, and can result in long-term complications. Most commonly associated with certain forms of juvenile idiopathic arthritis but may occur independent of arthritis. C26909 Uveitis C118468 Pediatric Rheumatology Terminology C116784 Circumscribed Morphea Circumscribed Morphea A type of morphea in which the lesions are circular or ovoid, and may be superficial or deep. The superficial lesions can have an indurated, waxy, ivory colored center with surrounding erythema or violaceous color during the active stage. Deep lesions can be sclerotic and depressed from underlying atrophy, and may show minimal skin color changes. When there are several (greater than or equal to 4), larger (greater than 3cm) lesions on two or more body areas this is classified as "generalized morphea". A type of morphea in which the lesions are circular or ovoid, and may be superficial or deep. The superficial lesions can have an indurated, waxy, ivory colored center with surrounding erythema or violaceous color during the active stage. Deep lesions can be sclerotic and depressed from underlying atrophy, and may show minimal skin color changes. When there are several (greater than or equal to 4), larger (greater than 3 cm) lesions on two or more body areas this is classified as "generalized morphea". C72069 Localized Scleroderma C118468 Pediatric Rheumatology Terminology C21527 Complement C3 Complement C3 C3 Complement C3 (1663 aa, ~187 kDa) is encoded by the human C3 gene. This protein plays a role in complement-mediated immunity. The component of the early complement cascade common to all three activation pathways. C3 is consumed by proteolysis upon activation. C3 levels are generally low when immune complex mediated diseases are active, such as systemic lupus erythematosus or post-streptococcal glomerulonephritis. C13236 Body Fluid or Substance C118468 Pediatric Rheumatology Terminology C70618 Complement Component-4 Complement C4 C4 Complement component-4 (1744 aa, ~193 kDa) is part of the complement cascade, which mediates the innate immune response to infection. The full length protein is a precursor that is secreted as a trimeric molecule containing an alpha, beta and gamma chain. When the complement cascade is initiated, the protein is cleaved. This proteolysis releases both the alpha chain, C4 anaphylatoxin, which stimulates inflammation, and a dimer of the beta and gamma chains, which mediates the interaction between the antigen-antibody complex and other complement components. An early component of the classical and mannose binding lectin (MBL) complement activation pathways. C4 is consumed by proteolysis upon activation of the classical and MBL pathways, but not the alternative pathway. C4 levels are generally low in active immune complex diseases mediated by classical or MBL activation, but not alternative activation, with the canonical example being systemic lupus erythematosus. C13236 Body Fluid or Substance C118468 Pediatric Rheumatology Terminology C4691 Complement Deficiency Complement Deficiency A broad classification for rare genetic disorders with mostly autosomal recessive patterns of inheritance. They are caused by the ineffective or decreased biosynthesis of complement components. Complement deficiencies may also be acquired acutely post-infection or chronically from co-morbid autoimmune disorders. If complement components are adequately synthesized, their rapid depletion may result in functional deficiencies. Clinical signs of inherited deficiencies present within the first decade of life and are consistent with the signs of recurrent systemic infection or immune complex disease. Complement deficiencies decrease the effectiveness of the humoral immune response. Of all the complement deficiencies, C3 deficiency is associated with the poorest prognosis since it presents at an early age, when susceptibility to recurrent infection is great. Deficiencies of C3 and of the classical activating pathway components: C1, C4, C2 also predispose to immune complex disease. A genetic deficiency of any component of the complement system, which may be associated with various pathologic conditions depending on the protein and activation pathway involved. C3101 Genetic Disorder C39725 Immunodeficiency C118468 Pediatric Rheumatology Terminology C70611 Complement Hemolytic Activity Assay Complement Hemolytic Activity Assay CH50|Total Hemolytic Activity Assay|Total Hemolytic Complement 50% A screening assay for circulating complement proteins in which diluted serum samples are added to antibody-coated erythrocytes and the percentage of cell lysis is measured. The values are expressed in hemolytic complement units per milliliter (CH50), the dilution of serum required to lyse 50 percent of the erythrocytes in the assay. A functional measurement of the ability of serum complement proteins to lyse target cells via classical activation that measures function of all complement components C1-C9. The CH50 is the reciprocal of the dilution of serum that induces lysis of 50% of target cells in a standardized assay. C25294 Laboratory Procedure C118468 Pediatric Rheumatology Terminology C85042 Complex Regional Pain Syndrome I Complex Regional Pain Syndrome I A neurologic condition characterized by burning pain, tenderness, swelling, and changes in the skin color and temperature of a body part or extremity that has no identifiable nerve injury; most pediatric patients present with this form. A neurologic condition characterized by burning pain, tenderness, swelling, and changes in the skin color and temperature of a body part or extremity that has no identifiable nerve injury; most pediatric patients present with this form. C119048 Pain Amplification Syndrome C118468 Pediatric Rheumatology Terminology Reflex Neurovascular Dystrophy|Reflex Sympathetic Dystrophy Syndrome C121572 Complex Regional Pain Syndrome II Complex Regional Pain Syndrome II Causalgia A neurologic condition characterized by burning pain, tenderness, swelling, and changes in the skin color and temperature of a body part or extremity associated with demonstrable nerve injury. This form is less frequent in pediatric patients. A neurologic condition characterized by burning pain, tenderness, swelling, and changes in the skin color and temperature of a body part or extremity associated with demonstrable nerve injury. This form is less frequent in pediatric patients. C119048 Pain Amplification Syndrome C118468 Pediatric Rheumatology Terminology C12374 Connective Tissue Connective Tissue The supporting or framework tissue of the animal body, formed of fibrous and ground substance with more or less numerous cells of various kinds; it is derived from the mesenchyme, and this in turn from the mesoderm; the varieties of connective tissue are: areolar or loose; adipose; dense, regular or irregular, white fibrous; elastic; mucous; and lymphoid tissue; cartilage; and bone; the blood and lymph may be regarded as connective tissues the ground substance of which is a liquid. Supporting tissue that surrounds other tissues and organs. Specialized connective tissue includes bone, cartilage, blood, and fat. C32221 Body Part C118468 Pediatric Rheumatology Terminology C26729 Connective Tissue Disorder Connective Tissue Disorder Disorder of Connective Tissue|Primary Disorder of Connective Tissue A non-neoplastic or neoplastic disorder that affects the connective tissue. A disorder characterized by abnormalities in one or more of the elements of the connective tissues, typically associated with genetic defects. C2991 Disease or Disorder C118468 Pediatric Rheumatology Terminology C2322 Corticosteroid Corticosteroid Hormones synthesized in the cortex of the adrenal gland and consist of two subclasses, glucocorticoids (carbohydrate regulation) and mineralocorticoids (electrolyte regulation). A group of naturally occurring chemical hormones produced by the adrenal cortex that regulate a large number of physiologic processes including stress responses, immune responses and inflammation, carbohydrate and protein metabolism, and electrolyte balance. The group includes glucocorticoids and mineralocorticoids. Synthetic analogues of naturally occurring hormones are also referred to as corticosteroids, and often simply called steroids. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C26733 Cranial Nerve Disorder Cranial Nerve Disorder Cranial Nerve Disease|Cranial Neuropathy|Disorder of Cranial Nerve A neoplastic or non-neoplastic disorder that affects one of the cranial nerves. Impaired or altered function of one or more of the cranial nerves. C27580 Peripheral Nervous System Disorder C118468 Pediatric Rheumatology Terminology C84657 Cryopyrin-Associated Periodic Syndrome Cryopyrin-Associated Periodic Syndrome CAPS|Cryopyrinopathy A group of autoinflammatory syndromes caused by mutations in the NLRP3 gene. Signs and symptoms include rash, fever, headache, joint pain, conjunctivitis, and weakness. The symptoms may exacerbate with exposure to cold weather. A family of autoinflammatory diseases caused by gain of function mutations in NLRP3, which encodes cryopyrin, resulting in inflammasome activation and enhanced IL-1 beta and IL-18 production. This results in clinical signs and symptoms of inflammation in the form of rash, fever, joint, neurologic and eye symptoms, with increased acute phase reactants. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C121362 Cutaneous Assessment Tool Cutaneous Assessment Tool CAT An assessment of a wide range of cutaneous manifestations reflecting activity and damage from idiopathic inflammatory myopathy in children and adults. An assessment of a wide range of cutaneous manifestations reflecting activity and damage from idiopathic inflammatory myopathy in children and adults. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121359 Cutaneous Dermatomyositis Disease Area and Severity Index Cutaneous Dermatomyositis Disease Area and Severity Index CDASI An instrument that measures activity and damage in the skin of dermatomyositis patients. An instrument that measures activity and damage in the skin of dermatomyositis patients. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C26819 Cutaneous Lupus Erythematosus Cutaneous Lupus Erythematosus An autoimmune disorder that manifests as different lupus-specific skin disorders; it can occur with systemic lupus erythematosus, or as a singular disease. An autoimmune disorder that manifests as different lupus-specific skin disorders; it can occur with systemic lupus erythematosus, or as a singular disease. C27153 Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C117295 Cutaneous Polyarteritis Nodosa Cutaneous Polyarteritis Nodosa Cutaneous PAN A form of vasculitis that involves small to medium size arteries of the dermis and subcutaneous tissue. The disorder manifests with tender subcutaneous nodules, livedo reticularis, cutaneous ulcers and necrosis; while internal organ involvement is typically spared, extra-cutaneous symptoms may be present. A form of vasculitis that involves small to medium size arteries of the dermis and subcutaneous tissue. The disorder manifests with tender subcutaneous nodules, livedo reticularis, cutaneous ulcers and necrosis; while internal organ involvement is typically spared, extra-cutaneous symptoms may be present. C26847 Polyarteritis Nodosa C118468 Pediatric Rheumatology Terminology C121326 Cyclic Citrullinated Peptide Antibody Cyclic Citrullinated Peptide Antibody ACPA|Anti-Keratin Antibody|Anti-Perinuclear Factor Antibody|CCP An autoantibody directed against peptides or proteins whose arginine residues have been enzymatically converted to citrulline. It is often associated with rheumatoid arthritis. An autoantibody directed against peptides or proteins whose arginine residues have been enzymatically converted to citrulline. It is often associated with rheumatoid arthritis. C16295 Antibody C118468 Pediatric Rheumatology Terminology C3820 Cyclic Neutropenia Cyclic Neutropenia CH|CN|Cyclic Hematopoiesis A hematologic disorder caused by a mutation in the ELANE (ELA2) gene; clinical manifestations include recurrent neutropenia with resultant susceptibility to infection leading to fever. A hematologic disorder caused by a mutation in the ELANE (ELA2) gene. Clinical manifestations include recurrent neutropenia with resultant susceptibility to infection leading to fever. C3101 Genetic Disorder C26323 Hematologic Disorder C118468 Pediatric Rheumatology Terminology C121651 Deep Circumscribed Morphea Deep Circumscribed Morphea Deep Morphea Circumscribed morphea in which the lesions are found in the deep dermis, panniculus, fascia, or superficial muscle. Circumscribed morphea in which the lesions are found in the deep dermis, panniculus, fascia, or superficial muscle. C116784 Circumscribed Morphea C118468 Pediatric Rheumatology Terminology C119056 Deficiency of the Interleukin-1 Receptor Antagonist Deficiency of the Interleukin-1 Receptor Antagonist Autoinflammatory Disease due to Interleukin-1 Receptor Antagonist Deficiency|DIRA An autoinflammatory disease caused by mutations in the IL1RN gene, which encodes the IL1 receptor antagonist. It presents in infancy, and is characterized by systemic inflammation, pustular rash, bone pain, sterile osteitis, and periostitis. An autoinflammatory disease caused by mutations in the IL1RN gene, which encodes the IL1 receptor antagonist. It presents in infancy, and is characterized by systemic inflammation, pustular rash, bone pain, sterile osteitis, and periostitis. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C119057 Deficiency of the Interleukin-36 Receptor Antagonist Deficiency of the Interleukin-36 Receptor Antagonist DITRA|Familial Generalized Pustular Psoriasis|PSORP An autoinflammatory disease caused by mutations in the IL36RN gene, which encodes the IL36 receptor antagonist. It is characterized by periodic fevers and psoriasiform rash. An autoinflammatory disease caused by mutations in the IL36RN gene, which encodes the IL36 receptor antagonist. It is characterized by periodic fevers and psoriasiform rash. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C121363 Dermatomyositis Skin Severity Index Dermatomyositis Skin Severity Index DSSI An assessment of disease activity in the skin of patients with dermatomyositis. An assessment of disease activity in the skin of patients with dermatomyositis. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C116791 Diffuse Cutaneous Systemic Sclerosis Diffuse Cutaneous Systemic Sclerosis dSSc|dcSSc A variant of systemic scleroderma characterized by sclerosis of the skin, Raynaud phenomenon, and organ involvement, including pulmonary fibrosis, renal disease, and gastrointestinal tract involvement. A variant of systemic scleroderma characterized by sclerosis of the skin, Raynaud phenomenon, and organ involvement, including pulmonary fibrosis, renal disease, and gastrointestinal tract involvement. C72070 Systemic Sclerosis C118468 Pediatric Rheumatology Terminology C26820 Discoid Lupus Erythematosus Discoid Lupus Erythematosus DLE A chronic, autoimmune skin condition that manifests with a red, scaling rash, most often found on the face, ears, and scalp; these lesions often lead to permanent scarring and dyspigmentation. Patients may have lesions with or without other symptoms or antibodies suggestive of systemic lupus erythematosus (SLE). A chronic, autoimmune skin condition that manifests with a red, scaling rash, most often found on the face, ears, and scalp; these lesions often lead to permanent scarring and dyspigmentation. Patients may have lesions with or without other symptoms or antibodies suggestive of systemic lupus erythematosus (SLE). C27153 Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C117105 Discoid Rash Discoid Rash Discoid Eruption A disc-shaped patch(es) of skin inflammation that is red, raised, and scaly; it typically develops on the face, scalp, and neck, and often leaves scars and dyspigmentation as a result of postinflammatory hyperpigmentation. A disc-shaped patch(es) of skin inflammation that is red, raised, and scaly; it typically develops on the face, scalp, and neck, and often leaves scars and dyspigmentation as a result of postinflammatory hyperpigmentation. C39594 Skin Rash C118468 Pediatric Rheumatology Terminology C121332 Disease-Modifying Antirheumatic Drug Disease-Modifying Antirheumatic Drug DMARD A pharmaceutical agent that is categorized by its ability to alter the progression of autoimmune disease. A pharmaceutical agent that is categorized by its ability to alter the progression of autoimmune disease. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C114354 Drug Induced Lupus Erythematosus Drug Induced Lupus Erythematosus Drug Induced Lupus|Drug-Induced Lupus An autoimmune disorder, similar to systemic lupus erythematosus, that is caused by certain drugs. A lupus-like condition which occurs after exposure to a specific pharmacologic agent and typically resolves after discontinuation of the agent. C3507 Immune System Disorder C118468 Pediatric Rheumatology Terminology C119037 Early Disseminated Lyme Disease Early Disseminated Lyme Disease An early stage of Lyme disease that may present days to weeks after a tick bite with relapsing signs of a red, concentrically-expanding rash (erythema migrans), facial palsy, meningismus, neuralgia, heart palpitations, and dizziness. An early stage of Lyme disease that may present days to weeks after a tick bite with relapsing signs of a red, concentrically-expanding rash (erythema migrans), facial palsy, meningismus, neuralgia, heart palpitations, and dizziness. C45161 Lyme Disease C118468 Pediatric Rheumatology Terminology C119022 Early Inflammatory Arthritis Early Inflammatory Arthritis Early stages of inflammatory arthritis, when the underlying diagnosis is uncertain, usually little or no joint damage has occurred. Treatment may alter the disease course (e.g., potentially avert progression to full RA or other diagnosis). Early stages of inflammatory arthritis, when the underlying diagnosis is uncertain, usually little or no joint damage has occurred. Treatment may alter the disease course (e.g., potentially avert progression to full RA or other diagnosis). C2883 Arthritis C118468 Pediatric Rheumatology Terminology C119036 Early Localized Lyme Disease Early Localized Lyme Disease An early stage of Lyme disease that may present within 3-30 days after a tick bite with a red, concentrically-expanding rash (erythema migrans), fatigue, chills, fever, headache, muscle and joint aches, and swollen lymph nodes. An early stage of Lyme disease that may present within 3-30 days after a tick bite with a red, concentrically-expanding rash (erythema migrans), fatigue, chills, fever, headache, muscle and joint aches, and swollen lymph nodes. C45161 Lyme Disease C118468 Pediatric Rheumatology Terminology C119045 Early Rheumatoid Arthritis Early Rheumatoid Arthritis Recent diagnosis of rheumatoid arthritis (usually within one to two years of onset) during which treatment may be more effective and possibly improve the disease course. Recent diagnosis of rheumatoid arthritis (usually within one to two years of onset) during which treatment may be more effective and possibly improve the disease course. C2884 Rheumatoid Arthritis C118468 Pediatric Rheumatology Terminology C119027 Easy Bruising Easy Bruising Appearance of skin bruises following perceived minimal contact or injury. Appearance of skin bruises following perceived minimal contact or injury. C100104 Sign or Symptom C118468 Pediatric Rheumatology Terminology C114470 Enthesitis Enthesitis Inflammation at the site of insertion of ligaments, tendons, and other fibrous structures into bone. Inflammation where ligaments, tendons, and other fibrous structures attach to bone. C107377 Musculoskeletal System Disorder C118468 Pediatric Rheumatology Terminology C119024 Enthesitis-Related Arthritis Enthesitis-Related Arthritis ERA|Enthesitis-Related JIA|Enthesitis-Related Juvenile Idiopathic Arthritis A category of juvenile idiopathic arthritis associated with arthritis and enthesitis, which may involve the axial skeleton. It is a form of juvenile spondyloarthritis. A category of juvenile idiopathic arthritis associated with arthritis and enthesitis, which may involve the axial skeleton. It is a form of juvenile spondyloarthritis. C114357 Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C112116 Eosinophilic Fasciitis Eosinophilic Fasciitis Shulman Syndrome Inflammation and induration of the fascia related to an accumulation of white blood cells, including eosinophils. An inflammatory disease of the fascia caused by infiltration of eosinophils, which manifests as progressive thickening of the tissues, often with accompanying erythema, pain, and induration. A classic finding is eosinophilia in the peripheral blood. C3371 Skin Disorder C118468 Pediatric Rheumatology Terminology C112189 Bulbar Conjunctivitis Episcleritis Bulbar Conjunctivitis Inflammation of the thin layer of tissue lining the sclera of the eye characterized by redness in the white portion of the eye. Inflammation of the episclera, the thin layer of tissue between the conjunctiva and the sclera. C3371 Skin Disorder C118468 Pediatric Rheumatology Terminology C119047 Erythema Migrans Erythema Migrans Bulls-Eye Rash|Erythema Chronicum Migrans A red, concentrically-expanding rash sometimes with central clearing, that is part of the first stage of Lyme disease caused by infection with Borrelia species. A red, concentrically-expanding rash sometimes with central clearing, that is part of the first stage of Lyme disease caused by infection with Borrelia species. C39594 Skin Rash C118468 Pediatric Rheumatology Terminology C74611 Erythrocyte Sedimentation Rate Measurement Erythrocyte Sedimentation Rate ESR|Sed Rate A quantitative measurement of the distance that red blood cells travel in one hour in a sample of unclotted blood. A quantitative measurement of the distance that red blood cells settle in one hour in a sample of unclotted blood. Used in the evaluation of systemic inflammation. C25294 Laboratory Procedure C118468 Pediatric Rheumatology Terminology C121339 European League Against Rheumatism Classification Criteria for Juvenile Chronic Arthritis European League Against Rheumatism Classification Criteria for Juvenile Chronic Arthritis EULAR Classification Criteria for Juvenile Chronic Arthritis A set of antiquated criteria used to assign children with chronic arthritis to one of six mutually exclusive categories. A set of antiquated criteria used to assign children with chronic arthritis to one of six mutually exclusive categories. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C119041 Extended Oligoarticular Juvenile Idiopathic Arthritis Extended Oligoarticular Juvenile Idiopathic Arthritis Extended Oligoarticular JIA Oligoarticular juvenile idiopathic arthritis that eventually involves more than four separate joints. Oligoarticular juvenile idiopathic arthritis that eventually involves more than four separate joints. C119032 Oligoarticular Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C119053 Familial Cold Autoinflammatory Syndrome Familial Cold Autoinflammatory Syndrome FCAS An autoinflammatory disease caused by mutations in the NLRP3 gene which encodes cryopyrin. It is characterized by short episodes of fever, rash, and arthralgia after exposure to cold or rapid decrease in temperature. An autoinflammatory disease caused by mutations in the NLRP3 gene which encodes cryopyrin. It is characterized by short episodes of fever, rash, and arthralgia after exposure to cold or rapid decrease in temperature. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C84707 Familial Mediterranean Fever Familial Mediterranean Fever FMF A usually autosomal recessive inherited inflammatory disorder caused by mutations in the MEFV gene. It is characterized by recurrent painful inflammatory attacks in the abdomen, joints, and chest. The inflammatory attacks are associated with fever. An autoinflammatory disease caused by MEFV gene mutations resulting in enhanced IL-1 beta production. This results in attacks of fever, rash, peritoneal, pleural, or pericardial serositis, and/or synovial inflammation along with increased acute phase reactants. Complications may include vasculitis or amyloidosis. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C87497 Fibromyalgia Fibromyalgia Fibromyalgia Syndrome A chronic disorder of unknown etiology characterized by pain, stiffness, and tenderness in the muscles of neck, shoulders, back, hips, arms, and legs. Other signs and symptoms include headaches, fatigue, sleep disturbances, and painful menstruation. Chronic, diffuse, non-inflammatory musculoskeletal pain disorder with characteristic tender points of neck, back, shoulders, hips, arms and legs, and associated with fatigue and sleep disturbance. Other symptoms may include headaches, abdominal discomfort, and depression/anxiety. It may also be associated with a primary rheumatic disorder. C107377 Musculoskeletal System Disorder C118468 Pediatric Rheumatology Terminology C116782 Generalized Morphea Generalized Morphea A type of morphea characterized by four or more plaques found in two or more anatomic locations. The plaques are indurated, generally well-delineated, and may include muscle atrophy in affected areas; there is no visceral involvement. A type of morphea characterized by four or more plaques found in two or more anatomic locations. The plaques are indurated, generally well-delineated, and may include muscle atrophy in affected areas; there is no visceral involvement. C26746 Scleroderma C118468 Pediatric Rheumatology Terminology C2323 Glucocorticoid Glucocorticoid A group of corticosteroids that affect carbohydrate metabolism (gluconeogenesis, liver glycogen deposition, elevation of blood sugar), inhibit corticotropin secretion, and possess pronounced anti-inflammatory activity. They also play a role in fat and protein metabolism, maintenance of arterial blood pressure, alteration of the connective tissue response to injury, reduction in the number of circulating lymphocytes, and functioning of the central nervous system. (MSH2002_06_01) A group of corticosteroids that affect carbohydrate metabolism, inhibit corticotropin secretion, and possess pronounced anti-inflammatory activity. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C3444 Wegener Granulomatosis Granulomatosis with Polyangiitis GPA|Wegener Granulomatosis|Wegener's Granulomatosis A rare, autoimmune, systemic medium and small size vasculitis. It is characterized by the formation of necrotizing granulomas in the respiratory tract, necrotizing angiitis, and glomerulonephritis. Systemic vasculitis affecting small to medium-sized vessels, characterized by necrotizing granulomas in the respiratory tract, necrotizing angiitis, glomerulonephritis with little to no immune complex deposition, and typically positive for antineutrophil cytoplasmic antibody staining in a cytoplasmic pattern (C-ANCA; antiproteinase 3). C26912 Vasculitis C118468 Pediatric Rheumatology Terminology C121546 Health Assessment Questionnaire Health Assessment Questionnaire HAQ|Stanford Health Assessment Questionnaire A comprehensive, self-report survey consisting of twenty questions intended as a functional measure of an individual's current ability to accomplish the activities of daily living. A comprehensive, self-report survey consisting of twenty questions intended as a functional measure of an individual's current ability to accomplish the activities of daily living. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C34665 Heart Block by ECG Finding Heart Block Conduction Block|Heart Block by ECG Finding An electrocardiographic finding of blocked cardiac electrical impulses along the fibers normally responsible for impulse conduction. An electrocardiographic finding of disturbance, delay, or interruption of cardiac electrical impulses along the fibers normally responsible for impulse conduction. C2881 Arrhythmia C118468 Pediatric Rheumatology Terminology C3090 Hematuria Hematuria Blood in the urine. Blood in the urine. C3149 Kidney Disease C118468 Pediatric Rheumatology Terminology C34963 Henoch-Schönlein Purpura Henoch-Schönlein Purpura HSP A systemic, usually self-limited immune complex vasculitis, characterized by immunoglobulin A deposition in the small vessels and kidneys. It is manifested with small hemorrhages in the skin, gastrointestinal symptoms, arthritis, and nephropathy. A systemic, usually self-limited immune complex vasculitis, characterized by immunoglobulin A deposition in the small vessels and kidneys. It is manifested with small hemorrhages in the skin, gastrointestinal symptoms, arthritis, and nephropathy. C2931 Cardiovascular Disorder C118468 Pediatric Rheumatology Terminology C29702 Histamine-2 Receptor Antagonist Histamine-2 Receptor Antagonist H2 Blocker Any agent that competitively binds to and blocks the histamine H2 receptors found in the stomach, heart, uterus, on vascular smooth muscle and in the central nervous system (CNS). Of particular clinical relevance is the blockage of H2 receptors found on the parietal cells of the stomach, which prevents gastric acid secretion. A pharmaceutical agent that blocks the binding of histamine to the type II histamine receptor, and is often used to decrease gastric acid production and to treat urticaria. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C116796 Idiopathic Inflammatory Myopathy Idiopathic Inflammatory Myopathy IIM|Idiopathic Inflammatory Myopathies An umbrella term for diseases which have chronic muscle inflammation and weakness of unknown etiology. The types of idiopathic inflammatory myopathy are further defined by either clinicopathologic criteria or by the presence of certain autoantibodies. An umbrella term for diseases which have chronic muscle inflammation and weakness of unknown etiology. The types of IIM are further defined by either clinicopathologic criteria or by the presence of certain autoantibodies. C101216 Myopathy C118468 Pediatric Rheumatology Terminology C2701 Therapeutic Immune Globulin Immune Globulin Gamma Globulin A preparation of plasma proteins derived from the pooled plasma of adult donors. Largely comprised of IgG antibodies, therapeutic immune globulin provides passive immunization by increasing the recipient's serum levels of circulating antibodies. IgG antibodies have multiple functions, including binding to and neutralizing bacterial toxins; opsonization of pathogens; activation of complement; and suppression of pathogenic cytokines and phagocytes through binding to CD5, interleukin-1a (IL-1a), interleukin 6 (IL-6), tumor necrosis factor-alpha (TNF-alpha), and T-cell receptors. Therapeutic immune globulin may diminish pathogenic mechanisms in some autoimmune diseases by binding to and inhibiting the activity of autoantibodies. (NCI04) A blood product derived from pooled IgG antibodies extracted from donor plasma delivered parenterally. It is used to treat multiple disorders, including immunodeficiencies, autoimmune disorders, and active infections. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C14139 Immunocompromised Immunocompromised Immunocompromised Host|Immunocompromised Patient|Immunosuppressed Host|Immunosuppressed Patient|Immunosuppressed A loss of any arm of immune functions, resulting in potential or actual increase in infections. This state may be reached secondary to specific genetic lesions, syndromes with unidentified or polygenic causes, acquired deficits from other disease states, or as result of therapy for other diseases or conditions. A loss of any arm of immune functions resulting in potential or actual increase in infections. This state may be reached due to specific genetic lesions, syndromes with unidentified or polygenic causes, acquired deficits from other disease states, or as result of therapy for other diseases or conditions. C3507 Immune System Disorder C118468 Pediatric Rheumatology Terminology C39725 Immunodeficiency Immunodeficiency Immunodeficient A disorder in which the immune system is unable to mount an adequate immune response. A state in which the immune system is compromised or absent. May be primary (congenital) or secondary (acquired). C3507 Immune System Disorder C118468 Pediatric Rheumatology Terminology C84786 Inclusion Body Myositis Inclusion Body Myositis An acquired or hereditary chronic inflammatory disorder of the muscles characterized by the morphologic finding of vacuoles and filamentous inclusions in the muscle tissues. A rare form of progressive muscle disease that is notable for cytoplasmic granules and vacuoles in the muscle with little inflammation, and is usually refractory to treatment. Usually symptoms begin after the age of fifty. C27578 Myositis C118468 Pediatric Rheumatology Terminology C121338 International League of Associations for Rheumatology Classification Criteria for Juvenile Idiopathic Arthritis International League of Associations for Rheumatology Classification Criteria for Juvenile Idiopathic Arthritis ILAR Classification Criteria for JIA A set of criteria used to assign children with chronic arthritis to one of the seven mutually exclusive categories of juvenile idiopathic arthritis (JIA), intended to be used for research purposes. A set of criteria used to assign children with chronic arthritis to one of the seven mutually exclusive categories of juvenile idiopathic arthritis (JIA), intended to be used for research purposes. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121335 International Myositis Assessment and Clinical Studies Group International Myositis Assessment and Clinical Studies Group IMACS A coalition of health care providers and researchers with experience and interest in the myositis syndromes. (adapted from NIEHS website) A coalition of health care providers and researchers with experience and interest in the myositis syndromes. (adapted from NIEHS website) C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121366 Intraarticular Corticosteroid Therapy Intraarticular Corticosteroid Intra-articular Corticosteroid Treatment with a corticosteroid formulation designed for and administered into a joint space. Treatment with a corticosteroid formulation designed for and administered into a joint space. C121367 Localized Corticosteroid C118468 Pediatric Rheumatology Terminology C121331 Intravenous Immunoglobulin Therapy Intravenous Immunoglobulin Gamma Globulin|IVIG|Immune Globulin The administration of a blood product derived from pooled IgG antibodies extracted from donor plasma delivered intravenously. It is used to treat multiple disorders, including immunodeficiencies, autoimmune disorders, and active infections. The administration of a blood product derived from pooled IgG antibodies extracted from donor plasma delivered intravenously. It is used to treat multiple disorders, including immunodeficiencies, autoimmune disorders, and active infections. C15986 Drug Therapy C118468 Pediatric Rheumatology Terminology C121354 Juvenile Arthritis Disease Activity Score Juvenile Arthritis Disease Activity Score JADAS A continuous measure of disease activity in juvenile arthritis related to inflammation. This validated measure has established cut points of minimal disease activity, inactive disease and remission. A continuous measure of disease activity in juvenile arthritis related to inflammation. This validated measure has established cut points of minimal disease activity, inactive disease and remission. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C27576 Childhood Dermatomyositis Juvenile Dermatomyositis Childhood Dermatomyositis|JDM An inflammatory myopathy of childhood resulting in muscle weakness, and associated with a characteristic skin rash. An inflammatory myopathy of childhood resulting in symmetric proximal muscle weakness, and associated with characteristic cutaneous findings, such as heliotrope rash or Gottron's papules. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C116797 Juvenile Dermatomyositis Sine Myositis Juvenile Dermatomyositis sine Myositis Juvenile Amyopathic Dermatomyositis A rare form of juvenile dermatomyositis that manifests with characteristic cutaneous findings for at least six months in the absence of any detectable muscle involvement. A rare form of juvenile dermatomyositis that manifests with characteristic cutaneous findings for at least six months in the absence of any detectable muscle involvement. C27576 Juvenile Dermatomyositis C118468 Pediatric Rheumatology Terminology C114357 Juvenile Idiopathic Arthritis Juvenile Idiopathic Arthritis JIA A group of chronic, inflammatory childhood disorders of unknown etiology that primarily involve joints. An umbrella term for arthritis of unknown etiology that begins before the sixteenth birthday, and persists for at least six weeks; other known conditions are excluded. Categories include: systemic, oligoarticular, polyarticular rheumatoid factor positive, polyarticular rheumatoid factor negative, psoriatic, enthesitis-related, or undifferentiated. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C116787 Juvenile Localized Scleroderma Juvenile Localized Scleroderma Localized scleroderma presenting before the age of eighteen. Localized scleroderma presenting before the age of eighteen. C72069 Localized Scleroderma C118468 Pediatric Rheumatology Terminology C117297 Juvenile Primary Fibromyalgia Syndrome Juvenile Primary Fibromyalgia Syndrome Chronic, diffuse, non-inflammatory musculoskeletal pain disorder associated with fatigue and sleep disturbance that can occur in childhood and adolescence. Tender points are not necessary to make the diagnosis, but if present may be less numerous than found in adults. Chronic, diffuse, non-inflammatory musculoskeletal pain disorder associated with fatigue and sleep disturbance that can occur in childhood and adolescence. Tender points are not necessary to make the diagnosis, but if present may be less numerous than found in adults. C107377 Musculoskeletal System Disorder C118468 Pediatric Rheumatology Terminology C27179 Juvenile Rheumatoid Arthritis Juvenile Rheumatoid Arthritis (AQ) An older, deprecated term that encompassed three major types of autoimmune or autoinflammatory arthritis in children: systemic-onset, pauciarticular, or polyarticular arthritis. The juvenile rheumatoid arthritis classification system has been replaced by the International League of Associations for Rheumatology (ILAR) juvenile idiopathic arthritis classification system. An older, deprecated term that encompassed three major types of autoimmune or autoinflammatory arthritis in children: systemic-onset, pauciarticular, or polyarticular arthritis. The juvenile rheumatoid arthritis classification system has been replaced by the International League of Associations for Rheumatology (ILAR) juvenile idiopathic arthritis classification system. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology JRA C114347 Juvenile Spondyloarthritis Juvenile Spondyloarthritis JSpA|Juvenile Spondyloarthropathy A group of chronic, inflammatory childhood diseases characterized by arthritis and enthesitis. This disorder can affect the axial skeleton in late childhood or young adulthood. Onset of spondyloarthritis before 18 years of age, and less commonly associated with axial disease. Encompasses the juvenile idiopathic arthritis categories of enthesitis-related arthritis and psoriatic arthritis, and includes many cases of undifferentiated arthritis. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C121365 Juvenile Spondyloarthritis Disease Activity Index Juvenile Spondyloarthritis Disease Activity Index JSpADA A set of criteria used to determine the activity of juvenile spondyloarthritis related to inflammation. A set of criteria used to determine the activity of juvenile spondyloarthritis related to inflammation. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C117103 Juvenile Systemic Sclerosis Juvenile Systemic Sclerosis JSSc|Juvenile Systemic Scleroderma Systemic sclerosis that is diagnosed in children. Juvenile systemic sclerosis is more likely to be of the overlap variant and presents with musculoskeletal involvement. Systemic sclerosis diagnosed in children. Juvenile systemic sclerosis is more likely to be of the overlap variant and present with musculoskeletal involvement. C72070 Systemic Sclerosis C118468 Pediatric Rheumatology Terminology C34825 Kawasaki Disease Kawasaki Disease Infantile Polyarteritis Nodosa|Mucocutaneous Lymph Node Syndrome A vasculitis characterized by inflammation of the arteries, particularly the coronary arteries. The vasculitis may lead to aneurysm formation and possibly, heart attacks. It affects young children who usually present with persistent high fever, redness of the mucous membranes of the mouth, redness of the palms and soles, skin rashes, lymphadenitis, and joint pain and swelling. Medium-sized vessel vasculitis seen primarily in young children with predilection for the coronary arteries. Diagnosis is based on clinical findings of persistent high fever, changes in lips and oral mucous membranes, erythema and edema of hands/feet and later desquamation, bilateral bulbar conjunctivitis, unilateral cervical adenopathy, polymorphous rash. C26912 Vasculitis C118468 Pediatric Rheumatology Terminology C34754 Kyphosis Kyphosis Abnormally increased curvature of the thoracic portion of the spine. Abnormally increased curvature of the thoracic portion of the spine. C110937 Musculoskeletal Finding C118468 Pediatric Rheumatology Terminology C119038 Late Disseminated Lyme Disease Late Disseminated Lyme Disease A late stage of Lyme disease that may present in untreated patients months to years after a tick bite manifested with intermittent bouts of arthralgia, arthritis and neurologic complaints. A late stage of Lyme disease that may present in untreated patients months to years after a tick bite manifested with intermittent bouts of arthralgia, arthritis and neurologic complaints. C45161 Lyme Disease C118468 Pediatric Rheumatology Terminology C70646 CREST Syndrome Limited Cutaneous Systemic Sclerosis CREST Syndrome|lSSc|lcSSc A variant of systemic sclerosis characterized by calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia. A variant of systemic scleroderma characterized by features such as calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia. C72070 Systemic Sclerosis C118468 Pediatric Rheumatology Terminology C116780 Linear Scleroderma Linear Scleroderma A type of localized scleroderma characterized by a long strip of indurated skin, which is typically found unilaterally on an arm or leg, and sometimes on the forehead or trunk. This disorder often affects the tissues beneath the skin, causing damage to bones, muscle or other organs. It can limit movement, alter growth, and disfigure the affected area. A type of localized scleroderma characterized by a long strip of indurated skin, which is typically found unilaterally on an arm or leg, and sometimes on the forehead or trunk. This disorder often affects the tissues beneath the skin, causing damage to bones, muscle or other organs. It can limit movement, alter growth, and disfigure the affected area. C26746 Scleroderma C118468 Pediatric Rheumatology Terminology C121367 Localized Corticosteroid Therapy Localized Corticosteroid Treatment with a corticosteroid formulation designed for delivery locally and specified by site of administration (intraarticular, topical, or inhaled). Treatment with a corticosteroid formulation designed for delivery locally and specified by site of administration (intraarticular, topical, or inhaled). C15986 Drug Therapy C118468 Pediatric Rheumatology Terminology C72069 Localized Scleroderma Localized Scleroderma Morphea A chronic disorder, possibly autoimmune, marked by excessive production of collagen which results in hardening and thickening of body tissues. Localized scleroderma typically affects the skin, with formation of patches or lines of thick and unyielding tissue; there can be muscle and underlying tissue involvement as well as occasional joint complications. The affected areas of skin can be restrictive and disfiguring. The shape, depth and location of the affected area is used to classify one of the four types of local scleroderma. An individual can have a combination of localized scleroderma types. An autoimmune disorder marked by excessive production of collagen resulting in hardening and thickening of skin, with formation of patches or lines of thick and unyielding tissue. There can also be musculoskeletal and underlying tissue involvement. The affected areas of skin can be restrictive and disfiguring. C26746 Scleroderma C118468 Pediatric Rheumatology Terminology C34787 Lordosis Lordosis Abnormally increased curvature of the lumbar portion of the spinal column. Abnormally increased curvature of the cervical or lumbar portion of the spine. C110937 Musculoskeletal Finding C118468 Pediatric Rheumatology Terminology C119995 Low Complement Low Complement A decreased amount of complement (e.g. C3, C4, CH50) as determined through quantitative measurement. May be due to a genetic deficiency or from overconsumption due to an infection or a co-morbid autoimmune disorder. A decreased amount of complement (e.g. C3, C4, CH50) as determined through quantitative measurement. May be due to a genetic deficiency or from overconsumption due to an infection or a co-morbid autoimmune disorder. C4691 Complement Deficiency C118468 Pediatric Rheumatology Terminology C119998 Low Natural Killer Cell Activity Low Natural Killer Cell Activity A decrease in the ability of peripheral mononuclear blood cells to lyse MHC I deficient target cells either due to low numbers of natural killer cells, or a reduction of their lytic activity. A decrease in the ability of peripheral mononuclear blood cells to lyse MHC I deficient target cells either due to low numbers of natural killer cells, or a reduction of their lytic activity. C3507 Immune System Disorder C118468 Pediatric Rheumatology Terminology C70620 Lupus Anticoagulant Antibody Lupus Anti-Coagulant Antibody LA|LAC|Lupus Anti-Coagulant Antibodies directed against plasma proteins (such as beta-2-glycoprotein I, prothrombin, or annexin V) bound to anionic phospholipids. The anticoagulant blocks in vitro assembly of the prothrombinase complex, resulting in prolongation of in vitro clotting assays such as the activated partial thromboplastin time, the Russell viper venom time, and others. Paradoxical to these changes which suggest impaired coagulation, patients with the lupus anticoagulant have an increase in thrombotic events. An antibody that results in a paradoxical prolongation of phospholipid-dependent clotting tests, such as the partial thromboplastin time, but in increases the propensity for thrombosis in the patient. C16295 Antibody C118468 Pediatric Rheumatology Terminology C27153 Lupus Erythematosus Lupus Erythematosus Lupus An autoimmune, connective tissue chronic inflammatory disorder affecting the skin, joints, kidneys, lungs, heart, and the peripheral blood cells. It is more commonly seen in women than men. Variants include discoid and systemic lupus erythematosus. An umbrella term referring to disease entities such as cutaneous, subacute cutaneous, discoid and systemic lupus erythematosus. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C117112 Lupus Erythematosus Tumidus Lupus Erythematosus Tumidus LET|Tumid Lupus Erythematosus A dermatologic manifestation of lupus involving edematous papules and plaques that are typically found on sun-exposed areas of the body. It most often resolves without scarring or pigmentation changes. A dermatologic manifestation of lupus involving edematous papules and plaques that are typically found on sun-exposed areas of the body. It most often resolves without scarring or pigmentation changes. C27153 Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C34789 Lupus Glomerulonephritis Lupus Glomerulonephritis Lupus Nephritis|SLE Nephritis Glomerulonephritis in the context of systemic lupus erythematosus. Glomerulonephritis in the context of systemic lupus erythematosus. C3149 Kidney Disease C118468 Pediatric Rheumatology Terminology C119029 Lupus Headache Lupus Headache A severe, persistent headache that occurs in patients with systemic lupus erythematosus, and is nonresponsive to narcotic analgesia. A severe, persistent headache that occurs in patients with systemic lupus erythematosus, and is nonresponsive to narcotic analgesia. C34661 Headache C118468 Pediatric Rheumatology Terminology C82884 Lupus Panniculitis Lupus Panniculitis A condition primarily affecting the subcutaneous adipose tissues, showing firm nodules that often resolve with lipoatrophy. There can be overlying changes of discoid lupus erythematosus. A skin biopsy showing lobular panniculitis with typical changes of lupus is needed to rule out other causes of panniculitis, including subcutaneous panniculitis-like T-cell lymphoma. A condition primarily affecting the subcutaneous adipose tissues, showing firm nodules that often resolve with lipoatrophy. There can be overlying changes of discoid lupus erythematosus. A skin biopsy showing lobular panniculitis with typical changes of lupus is needed to rule out other causes of panniculitis, including panniculitic lymphoma. C36281 Integumentary System Finding C118468 Pediatric Rheumatology Terminology C119026 Lyme Arthritis Lyme Arthritis Joint inflammation, most often affecting large joints, associated with Lyme disease, presenting months after infection. Joint inflammation, most often affecting large joints, associated with Lyme disease, presenting months after infection. C2883 Arthritis C118468 Pediatric Rheumatology Terminology C45161 Borrelia burgdorferi Infection Lyme Disease Borrelia burgdorferi Infection An infectious disease caused by the spirochete Borrelia burgdorferi. Early manifestations of infection may include fever, headache, fatigue, depression, and a characteristic skin rash called erythema migrans. Left untreated, late manifestations involving the joints, heart, and nervous system can occur. An infectious disease caused by the spirochete Borrelia burgdorferi. C26726 Infectious Disorder C118468 Pediatric Rheumatology Terminology C114471 Macrophage Activation Syndrome Macrophage Activation Syndrome MAS|Reactive Hemophagocytic Lymphohistiocytosis A complication of rheumatic disease that is caused by excessive activation and uncontrolled proliferation of T lymphocytes and well-differentiated macrophages. It is characterized by fever, pancytopenia, liver insufficiency, coagulopathy and neurologic symptoms. A potentially fatal complication of rheumatic disease characterized by fever, pancytopenia, hemophagocytosis, hyperferritinemia, liver insufficiency, coagulopathy and neurologic symptoms. C3507 Immune System Disorder C118468 Pediatric Rheumatology Terminology C117107 Macular Rash Macular Rash Macular Eruption An outbreak of small, flat, red spots on the skin. An outbreak of small, flat, red spots on the skin. C39594 Skin Rash C118468 Pediatric Rheumatology Terminology C119058 Majeed Syndrome Majeed Syndrome An autoinflammatory disease caused by mutations in the LPIN2 gene. It is characterized by early-onset chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and inflammatory dermatosis. An autoinflammatory disease caused by mutations in the LPIN2 gene. It is characterized by early-onset chronic recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia and inflammatory dermatosis. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C117106 Malar Rash Malar Rash Malar Eruption An erythematous eruption on the central face, which can be caused by a variety of different conditions. An erythematous eruption on the central face caused by a number of conditions. C39594 Skin Rash C118468 Pediatric Rheumatology Terminology C121360 Manual Muscle Testing Manual Muscle Testing MMT A graded muscle strength measurement performed as part of a physical examination without the need for specialized equipment. A graded muscle strength measurement performed as part of a physical examination without the need for specialized equipment. C18020 Diagnostic Procedure C118468 Pediatric Rheumatology Terminology C34807 Marfan Syndrome Marfan Syndrome Marfan's Syndrome A genetic syndrome inherited as an autosomal dominant trait. It is caused by mutations in the FBN1 gene. It is characterized by tall stature, elongated extremities, mitral valve prolapse, aortic dilatation, aortic dissection, and subluxation of the lens. An autosomal-dominant genetic syndrome caused by mutations in the FBN1 gene. It is characterized by tall stature, elongated extremities, pectus excavatum, mitral valve prolapse, aortic dilatation, aortic dissection, and subluxation of the lens. C3101 Genetic Disorder C97075 Hereditary Connective Tissue Disorder C118468 Pediatric Rheumatology Terminology C84890 Mevalonate Kinase Deficiency Mevalonate Kinase Deficiency HIDS|Hyperimmunoglobulin D with Periodic Fever Syndrome|MKD A very rare autosomal recessive disorder of cholesterol biosynthesis. It is caused by a deficiency of the enzyme mevalonate kinase, resulting in the accumulation of mevalonic acid in the urine. Signs and symptoms include psychomotor retardation, ataxia, recurrent fevers, skin rash, hepatosplenomegaly, and lymphadenopathy. An autoinflammatory disease caused by mutations in the mevalonate kinase (MVK) gene leading to partial deficiency. It is characterized by attacks of inflammation with fever, rash, lymphadenopathy, splenomegaly and serositis along with increased acute phase reactants. Some patients exhibit elevations in immunoglobulin D (hyperimmunoglobulin D). This disease is distinct from mevalonic aciduria caused by complete deficiency of MVK. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C70549 Microscopic Polyarteritis Microscopic Polyangiitis MPA A systemic necrotizing vasculitis that typically affects the small and medium-sized muscular arteries. In some cases, however, microscopic vessels are also affected (e.g., in the kidneys), a condition that has been called microscopic polyarteritis or polyangiitis; this disorder is felt to be more closely associated with Wegener granulomatosis than to classic polyarteritis nodosa. Systemic vasculitis affecting small vessels that is necrotizing and non-granulomatous, with little to no immune complex deposition. Clinically it is characterized by involvement of the kidneys, skin, lung, and peripheral nerves. Antineutrophil cytoplasmic antibody (anti-myeloperoxidase (anti-MPO)) is positive in most cases. C26912 Vasculitis C118468 Pediatric Rheumatology Terminology Microscopic Polyarteritis C84892 Mixed Connective Tissue Disease Mixed Connective Tissue Disease MCTD An autoimmune overlap syndrome characterized by the presence of symptoms of systemic lupus erythematosus, systemic scleroderma, and polymyositis. An autoimmune disorder which is associated with high titer anti-RNP antibody, and usually features Raynaud's Phenomenon and swollen fingers. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C116786 Mixed Morphea Mixed Morphea The presence of more than one variant of morphea in a single patient. The presence of more than one variant of morphea in a single patient. C72069 Localized Scleroderma C118468 Pediatric Rheumatology Terminology C119054 Muckle-Wells Syndrome Muckle-Wells Syndrome MWS An autoinflammatory disease caused by mutations in the NLRP3 gene which encodes cryopyrin. It is characterized by recurrent episodes of urticaria and fever which develop in infancy. It may lead to sensorineural hearing loss and/or amyloidosis. An autoinflammatory disease caused by mutations in the NLRP3 gene which encodes cryopyrin. It is characterized by recurrent episodes of urticaria and fever which develop in infancy. It may lead to sensorineural hearing loss and/or amyloidosis. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C118316 Mucosal Ulcer Mucosal Ulcer A circumscribed loss of integrity of the mucous membrane. A circumscribed loss of integrity of the mucous membrane. C3426 Ulcer C118468 Pediatric Rheumatology Terminology C27009 Myalgia Myalgia Muscle Pain Painful sensation originating from a muscle or group of muscles. Pain localized to muscle tissue. C3303 Pain C118468 Pediatric Rheumatology Terminology C34831 Myocarditis Myocarditis Inflammation of the muscle tissue of the heart. Inflammation of the myocardium. C3079 Heart Disorder C118468 Pediatric Rheumatology Terminology C27578 Myositis Myositis An inflammatory process affecting the skeletal muscles. Causes include infections, injuries, and autoimmune disorders. Inflammation affecting the skeletal muscles. C107377 Musculoskeletal System Disorder C118468 Pediatric Rheumatology Terminology C121358 Myositis Activities Profile Myositis Activities Profile MAP An assessment of disease-specific limitations of activities of daily living in patients with polymyositis and dermatomyositis. An assessment of disease-specific limitations of activities of daily living in patients with polymyositis and dermatomyositis. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121357 Myositis Damage Index Myositis Damage Index MDI A scoring system used to measure damage from myositis, complications of therapy, or other events. A scoring system used to measure damage from myositis, complications of therapy, or other events. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121356 Myositis Disease Activity Assessment Tool Myositis Disease Activity Assessment Tool MDAAT A tool that assesses disease activity of extramuscular organ systems and muscle to assess patients with adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis. A tool that assesses disease activity of extramuscular organ systems and muscle to assess patients with adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C119043 NALP12-Associated Hereditary Periodic Fever Syndrome NALP12-Associated Hereditary Periodic Fever Syndrome NLRP12-Associated Hereditary Periodic Fever Syndrome An autoinflammatory disease caused by mutations in the NLRP12 gene. It is characterized by periodic fevers beginning in the first year of life that are triggered by cold exposure. Episodes occur more than once per month. An autoinflammatory disease caused by mutations in the NLRP12 gene. It is characterized by periodic fevers beginning in the first year of life that are triggered by cold exposure. Episodes occur more than once per month. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C99236 Neonatal Lupus Erythematosus Neonatal Lupus Erythematosus A self-limited skin rash that appears in the neonatal period and usually resolves in four to six months after birth. It is caused by placental transfer of maternal autoantibodies, usually anti-Ro antibody. In a minority of cases, it is associated with congenital heart block, hepatitis, or thrombocytopenia. The mothers of the affected babies may be asymptomatic or suffer from systemic lupus erythematosus, Sjogren's syndrome, or rheumatoid arthritis. A disorder caused by placental transfer of maternal autoantibodies, usually anti-Ro (SSA) and/or anti-La (SSB), leading to fetal tissue damage associated with congenital heart block, and reversible manifestations such as skin rash, thrombocytopenia, and hepatitis. C98996 Neonatal Disorder C118468 Pediatric Rheumatology Terminology C116380 Chronic Infantile Neurological Cutaneous and Articular Syndrome Neonatal-Onset Multisystem Inflammatory Disease CINCA|Chronic Infantile Neurological Cutaneous and Articular Syndrome|NOMID A congenital autoinflammatory disorder that presents within a few days of birth and is characterized by a clinical triad of skin rash, chronic meningitis, and joint pain with recurrent fever and inflammation. An autoinflammatory disease caused by mutations in the NLRP3 gene which encodes cryopyrin. It typically presents during infancy and is characterized by fever, urticarial skin rash, aseptic meningitis, and widened epiphyses. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C121333 Non-Biologic Disease-Modifying Antirheumatic Drug Non-Biologic Disease-Modifying Antirheumatic Drug Non-Biologic DMARD A drug used to alter the progression of autoimmune disease that is not a biologic. A drug used to alter the progression of autoimmune disease that is not a biologic. C121332 Disease-Modifying Antirheumatic Drug C118468 Pediatric Rheumatology Terminology C119023 Nonerosive Arthritis Nonerosive Arthritis Non-Erosive Arthritis An inflammation of one or more joints, without loss of articular cartilage or destruction of subchondral bone. An inflammation of one or more joints, without loss of articular cartilage or destruction of subchondral bone. C2883 Arthritis C118468 Pediatric Rheumatology Terminology C257 Nonsteroidal Antiinflammatory Drug Nonsteroidal Antiinflammatory Drug NSAID Anti-inflammatory agents that are not steroids. In addition to anti-inflammatory actions, they have analgesic, antipyretic, and platelet-inhibitory actions. They are used primarily in the treatment of chronic arthritic conditions and certain soft tissue disorders associated with pain and inflammation. They act by blocking the synthesis of prostaglandins by inhibiting cyclooxygenase, which converts arachidonic acid to cyclic endoperoxides, precursors of prostaglandins. Inhibition of prostaglandin synthesis accounts for their analgesic, antipyretic, and platelet-inhibitory actions; other mechanisms may contribute to their anti-inflammatory effects. Certain NSAIDs also may inhibit lipoxygenase enzymes or phospholipase C or may modulate T-cell function. (MeSH) A class of medication typically used for its analgesic, antipyretic, anti-inflammatory, and/or platelet-inhibitory effects. Effects are classically exerted through inhibition of cyclooxygenase 1 and/or cyclooxygenase 2, though other pharmacologic effects may be involved. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C121351 Occupational Therapy Occupational Therapy OT Interventions and exercises intended to develop, recover, or maintain the ability of an individual to accomplish their activities of daily living. Interventions and exercises intended to develop, recover, or maintain the ability of an individual to accomplish their activities of daily living. C49236 Therapeutic Procedure C118468 Pediatric Rheumatology Terminology C119021 Oligoarticular Arthritis Oligoarticular Arthritis Oligoarthritis|Pauciarthritis An arthritis affecting fewer than five separate joints. An arthritis affecting fewer than five separate joints. C2883 Arthritis C118468 Pediatric Rheumatology Terminology C119032 Oligoarticular Juvenile Idiopathic Arthritis Oligoarticular Juvenile Idiopathic Arthritis Oligoarticular JIA|Pauciarticular JIA|Pauciarticular Juvenile Idiopathic Arthritis A category of juvenile idiopathic arthritis defined by the presence of arthritis affecting between one and four separate joints during the first six months of disease. A category of juvenile idiopathic arthritis defined by the presence of arthritis affecting between one and four separate joints during the first six months of disease. C114357 Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C117296 Orbital Myositis Orbital Myositis A rare form of myositis that affects only the orbital muscles. A rare form of myositis that only affects the orbital muscles. C27578 Myositis C118468 Pediatric Rheumatology Terminology C84978 Osteochondrodysplasia Osteochondrodysplasia Congenital Skeletal Dysplasia|Skeletal Dysplasia A term referring to disorders characterized by abnormalities in the development of bones and cartilage. A complex group of bone and cartilage disorders that result in abnormalities in the size and shape of the trunk, extremities, and/or skull. C89337 Congenital Musculoskeletal Defect C118468 Pediatric Rheumatology Terminology C116801 Overlap Syndrome Overlap Syndrome An autoimmune, connective tissue disorder in which the patient exhibits features from two or more diseases. These typically include systemic sclerosis, dermatomyositis, polymyositis, rheumatoid arthritis, systemic lupus erythematosus, and Sjogren syndrome; in pediatrics the respective pediatric entities are encountered. An autoimmune, connective tissue disorder in which the patient exhibits features from two or more diseases. These typically include systemic sclerosis, dermatomyositis, polymyositis, rheumatoid arthritis, systemic lupus erythematosus, and Sjogren syndrome; in pediatrics the respective pediatric entities are encountered. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C119048 Pain Amplification Syndrome Pain Amplification Syndrome Amplified Musculoskeletal Pain Syndrome|Chronic Widespread Pain A condition in which the nerves register normal stimuli (lack of tissue injury) as pain, resulting in feelings of intense pain with even minor sensory input. A condition in which the nerves register normal stimuli (e.g., stimuli not causing tissue injury) as pain, resulting in feelings of intense pain with even minor sensory input. C26835 Nervous System Disorder C3303 Pain C118468 Pediatric Rheumatology Terminology C3306 Pancreatitis Pancreatitis Inflammation of the pancreas. Inflammation of the pancreas. C2990 Gastrointestinal Disorder C118468 Pediatric Rheumatology Terminology C116785 Pansclerotic Morphea Pansclerotic Morphea A rare, aggressive form of morphea characterized by sclerosis of the dermis, fascia, and muscle over large parts of the body, resulting in contractures and immobility. A rare, aggressive form of morphea characterized by sclerosis of the dermis, fascia, and muscle over large parts of the body, resulting in contractures and immobility. C72069 Localized Scleroderma C118468 Pediatric Rheumatology Terminology C84989 Panuveitis Panuveitis A disorder characterized by inflammation of the entire uvea which includes the iris, ciliary body, and choroid. Causes include systemic infections, sarcoidosis, and cancers. Inflammation of all layers of the uvea. C26909 Uveitis C118468 Pediatric Rheumatology Terminology C121348 Parent Assessment of Overall Well-Being Parent Assessment of Overall Well-Being Parent Global Assessment A parent's estimation of the overall impact of a disease on their child at a given time. Typically scored on a numeric rating scale or visual analogue scale. A parent's estimation of the overall impact of a disease on their child at a given time. Typically scored on a numeric rating scale or visual analogue scale. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121347 Parent Global Assessment of Disease Activity Parent Global Assessment of Disease Activity A parent-scored instrument that estimates the level of activity of a disease at a given time taking into account all disease manifestations. Typically scored on a numeric rating scale or visual analogue scale, or may be recorded using descriptors such as remission, or low, moderate, or high disease activity. A parent-scored instrument that estimates the level of activity of a disease at a given time taking into account all disease manifestations. Typically scored on a numeric rating scale or visual analogue scale, or may be recorded using descriptors such as remission, or low, moderate, or high disease activity. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C114281 Parotitis Parotitis Inflammation of the parotid glands. Inflammation of the parotid glands. C118420 Otolaryngologic Disorder C118468 Pediatric Rheumatology Terminology C121350 Patient Assessment of Overall Well-Being Patient Assessment of Overall Well-Being Patient Global Assessment A patient's estimation of the overall impact of a disease on him or herself at a given time. Typically scored on a numeric rating scale or visual analogue scale. A patient's estimation of the overall impact of a disease on him or herself at a given time. Typically scored on a numeric rating scale or visual analogue scale. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121349 Patient Global Assessment of Disease Activity Patient Global Assessment of Disease Activity A patient-scored instrument that estimates the level of activity of a disease at a given time taking into account all disease manifestations. Typically scored on a numeric rating scale or visual analogue scale, or may be recorded using descriptors such as remission, or low, moderate, or high disease activity. A patient-scored instrument that estimates the level of activity of a disease at a given time taking into account all disease manifestations. Typically scored on a numeric rating scale or visual analogue scale, or may be recorded using descriptors such as remission, or low, moderate, or high disease activity. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C95401 Patient Reported Outcome Patient Reported Outcome PRO Information collected directly from a patient that typically includes subjective feelings regarding symptoms, functions in daily life, physical, mental, emotional, spiritual, and social well being, and satisfaction with his/her health care. Information collected directly from a patient that typically includes subjective feelings regarding symptoms, functions in daily life, physical, mental, emotional, spiritual, and social well being, and satisfaction with his/her health care. C3367 Finding C118468 Pediatric Rheumatology Terminology C121345 Patient-Reported Outcomes Measurement Information System Measures Patient-Reported Outcomes Measurement Information System Measures PROMIS Measures Psychometrically validated measures designed for precise and efficient self-report measurement of physical, mental, and social health across chronic conditions. Items can be administered in static (short-form) or dynamic form (as computer adaptive tests). Psychometrically validated measures developed using item response theory and designed for precise and efficient self-report measurement of physical, mental, and social health across chronic conditions. Items can be administered in static (short-form) or dynamic form (as computer adaptive tests). C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C61279 Oligoarticular Still Disease Pauciarticular Juvenile Rheumatoid Arthritis (AQ) Juvenile rheumatoid arthritis affecting four or fewer joints, usually asymmetrically. The most commonly affected joints are the knee, elbow, wrist, and ankle. An older, deprecated term for a category of juvenile rheumatoid arthritis that affects fewer than five joints; it most commonly affects the large joints, and is most often asymmetric. C27179 Juvenile Rheumatoid Arthritis (AQ) C118468 Pediatric Rheumatology Terminology Oligoarticular JRA|Oligoarticular Juvenile Rheumatoid Arthritis|Pauciarticular JRA C119996 Pediatric Osteoporosis Pediatric Osteoporosis The finding of one or more vertebral compression (crush) fractures in the absence of local disease or high-energy trauma or the presence of both a clinically significant fracture history and BMD Z-score less than or equal to -2.0. A clinically significant fracture history is one or more of the following: 1) two or more long bone fractures by age ten years; 2) three or more long bone fractures at any age up to age nineteen years. (from 2013 International Society for Clinical Densitometry definition of Osteoporosis-Pediatric) The finding of one or more vertebral compression (crush) fractures in the absence of local disease or high-energy trauma or the presence of both a clinically significant fracture history and BMD Z-score less than or equal to -2.0. A clinically significant fracture history is one or more of the following: 1) two or more long bone fractures by age ten years; 2) three or more long bone fractures at any age up to age nineteen years. (from 2013 International Society for Clinical Densitometry definition of Osteoporosis-Pediatric) C107377 Musculoskeletal System Disorder C118468 Pediatric Rheumatology Terminology C78652 Pediatric Quality of Life Inventory Pediatric Quality of Life Inventory PedsQL A parent report or patient self report for measuring health-related quality of life (HRQOL) in healthy children and adolescents and those with acute and chronic health conditions. The PedsQL Measurement Model uses a modular approach that integrates both generic core scales and disease-specific modules into one measurement system. (Copyright 1998-2008 James W. Varni, Ph.D.) A copyrighted measurement model that integrates both generic core scales and disease-specific modules to assess health-related quality of life for healthy children and those with acute and chronic conditions. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121342 Pediatric Vasculitis Activity Score Pediatric Vasculitis Activity Score PVAS A set of criteria used to determine the activity of childhood vasculitis. A set of criteria used to determine the activity of childhood vasculitis. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C34915 Pericarditis Pericarditis An inflammatory process affecting the pericardium. Inflammation of the pericardium, frequently manifested as chest pain, friction rub, pericardial effusion, or ST segment elevation on EKG. C101327 Pericardial Anomaly C118468 Pediatric Rheumatology Terminology C118240 Periodic Fever Syndrome Periodic Fever Syndrome Fevers of unknown etiology recurring over months or years. An umbrella term for disorders characterized by persistent, episodic fevers with variable clinical manifestations. They do not have an infectious etiology and lack autoantibodies or autoreactive T cells. Some are autoinflammatory in origin. C9229 Systemic Disorder C118468 Pediatric Rheumatology Terminology C116917 Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome Marshall Syndrome|PFAPA Syndrome An autoinflammatory syndrome of childhood which often resolves in adolescence, and is characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. The fever cycle generally occurs every three to five weeks, and during the interim periods the child appears healthy; diagnosis is exclusionary. An autoinflammatory disorder of childhood characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Flares with high fever alternate with totally asymptomatic periods. It often resolves in adolescence. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C119734 Peripheral Neuropathy Peripheral Neuropathy Peripheral Nerve Disorder A disorder affecting the peripheral nervous system. It manifests with pain, tingling, numbness, and muscle weakness. It may be the result of physical injury, toxic substances, viral diseases, diabetes, renal failure, cancer, and drugs. A disorder affecting one or more peripheral nerves. It is manifested with pain, tingling, numbness, and/ or muscle weakness. Causes may include physical injury, toxic substances, viral infection and systemic illness. C27580 Peripheral Nervous System Disorder C118468 Pediatric Rheumatology Terminology C26849 Peritonitis Peritonitis Inflammation of the peritoneum due to infection by bacteria or fungi. Causes include liver disease, perforation of the gastrointestinal tract or biliary tract, and peritoneal dialysis. Patients usually present with abdominal pain and tenderness, fever, chills, and nausea and vomiting. It is an emergency medical condition that requires prompt medical attention and treatment. Inflammation of the peritoneum. C2990 Gastrointestinal Disorder C118468 Pediatric Rheumatology Terminology C119040 Persistent Oligoarticular Juvenile Idiopathic Arthritis Persistent Oligoarticular Juvenile Idiopathic Arthritis Persistent Oligoarticular JIA Oligoarticular juvenile idiopathic arthritis that never involves more than four separate joints after six months. Oligoarticular juvenile idiopathic arthritis that never involves more than four separate joints after six months. C119032 Oligoarticular Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C15302 Physical Therapy Physical Therapy PT|Physiotherapy The treatment of deformity or disease through mechanical force and movement, including both active and passive exercises, intended to ameliorate injury, increase mobility, and improve overall function. The use of exercises and physical activity to restore movement, reduce pain, strengthen muscles, and improve balance and posture in order to restore the ability to function. Therapists provide education and suggest activity modification suited to an individual. C49236 Therapeutic Procedure C118468 Pediatric Rheumatology Terminology C121346 Physician Global Assessment of Disease Activity Physician Global Assessment of Disease Activity MD Global|PGA|Physician's Global Assessment A physician or clinician-scored instrument that estimates the level of activity of a disease at a given time taking into account all disease manifestations. Typically scored on a numeric rating scale or visual analogue scale, or may be recorded using descriptors such as remission, or low, moderate, or high disease activity. A physician or clinician-scored instrument that estimates the level of activity of a disease at a given time taking into account all disease manifestations. Typically scored on a numeric rating scale or visual analogue scale, or may be recorded using descriptors such as remission, or low, moderate, or high disease activity. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C15304 Plasmapheresis Plasmapheresis Plasma Exchange A procedure that collects plasma from the peripheral blood while returning the rest to the donor. A procedure that collects plasma from the peripheral blood while returning the rest to the donor's circulation. C15191 Apheresis C118468 Pediatric Rheumatology Terminology C3331 Pleural Effusion Pleural Effusion Increased amounts of fluid within the pleural cavity. Symptoms include shortness of breath, cough, and chest pain. It is usually caused by lung infections, congestive heart failure, pleural and lung tumors, connective tissue disorders, and trauma. An accumulation of fluid in the pleural cavity. C26871 Respiratory System Disorder C118468 Pediatric Rheumatology Terminology C26860 Pleuritis Pleuritis Pleurisy Inflammation of the pleura. It is usually caused by infections. Chest pain while breathing or coughing is the presenting symptom. Inflammation of the pleura. C26871 Respiratory System Disorder C118468 Pediatric Rheumatology Terminology C113159 Pneumonitis Pneumonitis An inflammatory process affecting the lung parenchyma. It is a milder form of lung inflammation compared to pneumonia. Inflammation of the lung parenchyma. C26871 Respiratory System Disorder C118468 Pediatric Rheumatology Terminology C26847 Polyarteritis Nodosa Polyarteritis Nodosa Classic Polyarteritis Nodosa|PAN|Panarteritis Nodosa|Periarteritis Nodosa A necrotizing vasculitis that affects medium-sized arteries, it is usually idiopathic, and may be triggered by viral infections (Hepatitis B, also others). A multisystem disease, it most commonly affects skin (nodules, livedo reticularis) and peripheral nerves; higher morbidity is associated with gastrointestinal, central nervous system, cardiac, and/or other organ involvement. Anti-neutrophil cytoplasmic antibodies (ANCA) are typically negative. A necrotizing vasculitis that affects medium-sized arteries, it is usually idiopathic, and may be triggered by viral infections (Hepatitis B, also others). A multisystem disease, it most commonly affects skin (nodules, livedo reticularis) and peripheral nerves; higher morbidity is associated with gastrointestinal, central nervous system, cardiac, and/or other organ involvement. Anti-neutrophil cytoplasmic antibodies (ANCA) are typically negative. C26912 Vasculitis C118468 Pediatric Rheumatology Terminology C26996 Polyarticular Arthritis Polyarticular Arthritis Polyarthritis An arthritis affecting five or more separate joints. An arthritis affecting five or more separate joints. C2883 Arthritis C118468 Pediatric Rheumatology Terminology C119033 Polyarticular Juvenile Idiopathic Arthritis, Rheumatoid Factor Negative Polyarticular Juvenile Idiopathic Arthritis, Rheumatoid Factor Negative Polyarticular JIA, RF-|Polyarticular Juvenile Idiopathic Arthritis, RF- A category of juvenile idiopathic arthritis defined by the presence of arthritis affecting five or more separate joints during the first six months of disease, with negative serologic testing for rheumatoid factor. A category of juvenile idiopathic arthritis defined by the presence of arthritis affecting five or more separate joints during the first six months of disease, with negative serologic testing for rheumatoid factor. C114357 Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C119034 Polyarticular Juvenile Idiopathic Arthritis, Rheumatoid Factor Positive Polyarticular Juvenile Idiopathic Arthritis, Rheumatoid Factor Positive Polyarticular JIA, RF+|Polyarticular Juvenile Idiopathic Arthritis, RF+ A category of juvenile idiopathic arthritis defined by the presence of arthritis affecting five or more separate joints during the first six months of disease, with positive serologic testing for rheumatoid factor. A category of juvenile idiopathic arthritis defined by the presence of arthritis affecting five or more separate joints during the first six months of disease, with positive serologic testing for rheumatoid factor. C114357 Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C61280 Polyarticular Still Disease Polyarticular Juvenile Rheumatoid Arthritis (AQ) Juvenile rheumatoid arthritis affecting multiple joints, usually symmetrically. It may be associated with low grade fever, anemia, and weight loss. Patients usually test negative for rheumatoid factor. An older, deprecated term for a category of juvenile rheumatoid arthritis that affects five or more joints. A subset of patients are rheumatoid factor positive. C27179 Juvenile Rheumatoid Arthritis (AQ) C118468 Pediatric Rheumatology Terminology Polyarticular JRA C26925 Polymyositis Polymyositis An idiopathic inflammatory disorder affecting the muscles. It presents with symmetrical proximal muscle weakness and elevated skeletal muscle enzymes. An inflammatory disorder affecting the muscles. It presents with symmetrical proximal muscle weakness. Complications include distal weakness, thickening skin of hands and interstitial lung disease. Juvenile polymyositis is a rare early onset form of the disease. C27578 Myositis C118468 Pediatric Rheumatology Terminology C119025 Post-Streptococcal Arthritis Post-Streptococcal Arthritis PSRA|Post-Streptococcal Reactive Arthritis The occurrence of arthritis after infection with group A streptococcus. It is non-migratory, protracted in course, and poorly responsive to anti-inflammatory agents. (from Textbook of Pediatric Rheumatology, 6th ed. Cassidy, Petty, Laxer, and Lindsley) The occurrence of arthritis after infection with group A streptococcus. It is non-migratory, protracted in course, and poorly responsive to anti-inflammatory agents. (from Textbook of Pediatric Rheumatology, 6th edition, Cassidy, Petty, Laxer, and Lindsley) C2883 Arthritis C118468 Pediatric Rheumatology Terminology C119039 Post-Treatment Lyme Disease Syndrome Post-Treatment Lyme Disease Syndrome Chronic Lyme Disease|PTLDS A constellation of symptoms lasting months to years reported by some Lyme disease patients who had undergone previous antibiotic therapy. A constellation of symptoms lasting months to years reported by some Lyme disease patients who had undergone previous antibiotic therapy. C45161 Lyme Disease C118468 Pediatric Rheumatology Terminology C35111 Posterior Uveitis Posterior Uveitis An inflammatory process that affects the choroid. Inflammation of the posterior uveal tract. C26909 Uveitis C118468 Pediatric Rheumatology Terminology C116360 Primary Raynaud Phenomenon Primary Raynaud Phenomenon Primary Raynaud's Phenomenon Raynaud phenomenon without a known underlying autoimmune disorder. Raynaud phenomenon without a known underlying autoimmune disorder. C50724 Raynaud Phenomenon C118468 Pediatric Rheumatology Terminology Primary Raynaud Disease|Primary Raynaud's Disease C116985 Primary Sjogren Syndrome Primary Sjogren Syndrome Primary Sjogren's syndrome Sjogren syndrome without a concomitant systemic autoimmune disorder. Sjogren syndrome without an associated systemic autoimmune disorder. C26883 Sjogren Syndrome C118468 Pediatric Rheumatology Terminology C116916 Progressive Hemifacial Atrophy Progressive Hemifacial Atrophy Parry-Romberg Syndrome A rare disorder of unknown etiology, which is characterized by slowly progressive, unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures. Neurological, ocular, and oral symptoms are also often seen, including migraines, trigeminal neuralgia, enophthalmos, and dental and gingival abnormalities. A rare disorder characterized by slowly progressive, unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures. C107377 Musculoskeletal System Disorder C118468 Pediatric Rheumatology Terminology C38012 Proteinuria Proteinuria The presence of abnormal amounts of protein in the urine. The presence of abnormal amounts of protein in the urine. C36292 Laboratory Test Result C118468 Pediatric Rheumatology Terminology C29723 Proton Pump Inhibitor Proton Pump Inhibitor PPI Any one of the substituted benzimidazole compounds with anti-ulcer activity. A proton pump inhibitor is orally administered in the neutrally charged, inactive form and, upon entering the acidic environment of the parietal cell, gets protonated and converted into its active form. The active form will covalently and irreversibly bind to the proton pump, the H+/K+-ATPase, located on the surface of parietal cells. This leads to an inhibition of the transport of hydrogen ions, in exchange for potassium ions, into the gastric lumen and results in an inhibition of gastric acid secretion. A pharmaceutical agent that inhibits the transport of hydrogen ions, in exchange for potassium ions, into the gastric lumen and results in an inhibition of gastric acid secretion. These drugs are used for their anti-ulcer activity. C1909 Pharmacologic Substance C118468 Pediatric Rheumatology Terminology C61277 Psoriatic Arthritis Psoriatic Arthritis Arthritis Psoriatica Joint inflammation associated with psoriasis. An inflammatory arthritis typically associated with dactylitis, nail dystrophy, and the absence of rheumatoid factor in an individual with psoriasis or a family history of psoriasis. C2883 Arthritis C118468 Pediatric Rheumatology Terminology Psoriatic Arthropathy C119044 Psoriatic Juvenile Idiopathic Arthritis Psoriatic Juvenile Idiopathic Arthritis Psoriatic JIA|psJIA A category of juvenile idiopathic arthritis defined by the presence of arthritis with a personal or family history of psoriasis, and features such as dactylitis and nail dystrophy. A category of juvenile idiopathic arthritis defined by the presence of arthritis with a personal or family history of psoriasis, and features such as dactylitis and nail dystrophy. C114357 Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C78787 Purpura Purpura A small blood vessel hemorrhage into the skin and/or mucous membranes. Newer lesions appear reddish in color. Older lesions are usually a darker purple color and eventually become a brownish-yellow color. Small blood vessel hemorrhage into the skin and/or mucous membranes presenting as red or purplish patches. C36281 Integumentary System Finding C118468 Pediatric Rheumatology Terminology C119055 Pyogenic Arthritis, Pyoderma Gangrenosum and Acne Pyogenic Arthritis, Pyoderma Gangrenosum and Acne PAPA An autoinflammatory disease caused by mutations in the PSTPIP1 gene. It is characterized by episodes of destructive arthritis, ulcerative skin lesions and cystic acne. An autoinflammatory disease caused by mutations in the PSTPIP1 gene. It is characterized by episodes of destructive arthritis, ulcerative skin lesions and cystic acne. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C119028 Pyuria Pyuria The presence of excessive white blood cells in the urine as determined by urinalysis. The presence of excessive white blood cells in the urine as determined by urinalysis. C36292 Laboratory Test Result C118468 Pediatric Rheumatology Terminology C121361 Quantitative Muscle Testing Quantitative Muscle Testing QMT Measurement of maximum isometric force generation from a muscle group; performed with specialized equipment. Measurement of maximum isometric force generation from a muscle group; performed with specialized equipment. C18020 Diagnostic Procedure C118468 Pediatric Rheumatology Terminology C50724 Raynaud Phenomenon Raynaud Phenomenon Raynaud's Phenomenon An episodic vasoconstriction resulting in discoloration of the skin and pain in the affected areas, often involving fingers or toes. Classically associated with triphasic color changes (white, blue, red) but may be biphasic. Often occurs in response to cold temperatures or emotional stress. May be primary or secondary to an underlying autoimmune disease. An episodic vasoconstriction resulting in discoloration of the skin and pain in the affected areas, often involving fingers or toes. Classically associated with triphasic color changes (white, blue, red) but may be biphasic. Often occurs in response to cold temperatures or emotional stress. May be primary or secondary to an underlying autoimmune disease. C35117 Vascular Disorder C118468 Pediatric Rheumatology Terminology Raynaud Disease|Raynaud's Disease C115993 Retinitis Retinitis Inflammation of the retina. Inflammation of the retina. C26875 Retinal Disorder C118468 Pediatric Rheumatology Terminology C27204 Rheumatologic Disorder Rheumatic Disease Collagen Vascular Disease|Rheumatologic Disorder Inflammatory and degenerative diseases of connective tissue structures, such as arthritis. An umbrella term used to encompass disorders characterized by autoimmunity, autoinflammatory processes, joint or connective tissue inflammation or degeneration. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology Connective Tissue Disease|Inflammatory Rheumatism C34984 Rheumatic Fever Rheumatic Fever ARF|Acute Rheumatic Fever An inflammatory disorder that follows infection with group A streptococcus. It affects the heart, joints, and subcutaneous tissues. It manifests with pericarditis, heart murmur, congestive heart failure, polyarthritis, subcutaneous nodules, and erythema marginatum. It is characterized by the formation of granulomatous lesions called Aschoff bodies usually in the heart tissue. An inflammatory disorder that follows infection with group A streptococcus. It may manifest with carditis, migratory polyarthritis, subcutaneous nodules, erythema marginatum, and chorea. C26726 Infectious Disorder C118468 Pediatric Rheumatology Terminology C2884 Rheumatoid Arthritis Rheumatoid Arthritis RA A chronic, systemic autoimmune disorder characterized by inflammation in the synovial membranes and articular surfaces. It manifests primarily as a symmetric, erosive polyarthritis that spares the axial skeleton and is typically associated with the presence in the serum of rheumatoid factor. A chronic, inflammatory condition manifesting primarily as a symmetric, erosive, polyarthritis that spares the axial skeleton and is typically associated with rheumatoid factor and anti-citrullinated protein antibodies. C2883 Arthritis C118468 Pediatric Rheumatology Terminology C805 Rheumatoid Factor Rheumatoid Factor RA Factor|RF Antibodies in the serum of individuals with rheumatoid arthritis that react with antigenic determinants or immunoglobulins that enhance agglutination of suspended particles coated with pooled human gamma-globulin. Rheumatoid factors also occur in other autoimmune and certain infectious diseases. Antibodies that bind other immunoglobulins frequently seen in rheumatoid arthritis but can be found in other autoimmune diseases. C16295 Antibody C118468 Pediatric Rheumatology Terminology C119030 Rheumatoid Nodule Rheumatoid Nodule A well circumscribed lump of tissue, firm to touch, typically non-tender, predominantly occurring as a cutaneous manifestation of rheumatoid arthritis localized to extensor surfaces near joints, but may be internal. A well circumscribed lump of tissue, firm to touch, typically non-tender, predominantly occurring as a cutaneous manifestation of rheumatoid arthritis localized to extensor surfaces near joints, but may be internal. C100104 Sign or Symptom C118468 Pediatric Rheumatology Terminology C34995 Sarcoidosis Sarcoidosis Sarcoid An idiopathic inflammatory disorder characterized by the formation of non-necrotizing epithelioid granulomas which contain giant cells. It usually affects the lungs, lymph nodes, liver, and skin. Cardiac involvement is also possible. An inflammatory disorder characterized by the formation of non-necrotizing granulomas often associated with multi-nucleated giant cells within affected organs. Virtually all organs may be affected; however, it often affects the lungs, lymph nodes, liver, synovium, skin, heart, and uveal tract. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C119046 Scleritis Scleritis Inflammation of the sclera. Inflammation of the sclera. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C116781 Scleroderma en Coup de Sabre Scleroderma en Coup de Sabre Frontal Linear Scleroderma A type of linear scleroderma characterized by a linear, colorless, atrophied band across the forehead or scalp. This disorder can affect the tissues under or near the lesion including brain, bone and eyes. A type of linear scleroderma characterized by a linear, colorless, atrophied band across the forehead or scalp. This disorder can affect the tissues under or near the lesion including brain, bone and eyes. C116780 Linear Scleroderma C118468 Pediatric Rheumatology Terminology C119999 Scleroderma Renal Crisis Scleroderma Renal Crisis The onset of acute renal failure, normally coupled with marked hypertension in a patient with scleroderma. The onset of acute renal failure, normally coupled with marked hypertension in a patient with scleroderma. C3149 Kidney Disease C118468 Pediatric Rheumatology Terminology C78603 Scoliosis Scoliosis A congenital or acquired spinal deformity characterized by lateral curvature of the spine. A congenital or acquired spinal deformity characterized by lateral curvature of the spine. C107377 Musculoskeletal System Disorder C99064 Spinal Deformity C118468 Pediatric Rheumatology Terminology C116359 Secondary Raynaud Phenomenon Secondary Raynaud Phenomenon Raynaud Syndrome|Raynaud's Syndrome|Secondary Raynaud's Phenomenon Raynaud phenomenon associated with an underlying autoimmune disorder. Raynaud phenomenon associated with an underlying autoimmune disorder. C50724 Raynaud Phenomenon C118468 Pediatric Rheumatology Terminology Secondary Raynaud Disease|Secondary Raynaud's Disease C116986 Secondary Sjogren Syndrome Secondary Sjogren Syndrome Secondary Sjogren's syndrome Sjogren syndrome that occurs with another systemic autoimmune disorder, such as systemic lupus erythematosus or rheumatoid arthritis. Sjogren syndrome associated with another systemic autoimmune disorder, such as systemic lupus erythematosus or rheumatoid arthritis. C26883 Sjogren Syndrome C118468 Pediatric Rheumatology Terminology C119994 Seronegative Seronegative A laboratory result which shows no presence of antibodies for which sera are being tested. In rheumatology this typically refers to absence of rheumatoid factor. A laboratory result which shows no presence of antibodies for which sera are being tested. In rheumatology this typically refers to absence of rheumatoid factor. C36292 Laboratory Test Result C118468 Pediatric Rheumatology Terminology C70428 Serositis Serositis Inflammation of a serous membrane. Inflammation of the serous tissues; specifically, it may refer to inflammation of the pleura, pericardium, or peritoneum. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C70647 Sicca Syndrome Sicca Syndrome A constellation of symptoms that include abnormal dryness of the mouth, eyes and other mucous membranes. The condition is seen in patients with Sjogren syndrome, sarcoidosis, amyloidosis, and deficiencies of vitamins A and C. A syndrome of dry eyes and/or dry mouth due to autoimmune destruction of the lacrimal and salivary glands, respectively. It is one of the major manifestations of Sjogren syndrome. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C26883 Sjogren Syndrome Sjogren Syndrome Sjogren's Syndrome An autoimmune disorder affecting the salivary and lacrimal glands. Morphologically, it is characterized by the presence of lymphocytic and plasmacytic infiltrates which cause destruction of these glands. It results in dry mouth and dry eyes. It may be associated with the presence of other autoimmune disorders, including rheumatoid arthritis and lupus erythematosus. Autoimmune epithelial inflammation often affecting the salivary and lacrimal glands (causing dry mouth and dry eyes) with potential extraglandular manifestations. In children, it most commonly presents with recurrent parotitis. It may occur alone (primary) or in association with another autoimmune disease (secondary). C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C39594 Skin Rash Skin Rash Rash|Skin Eruption Any change in the skin which affects its appearance or texture. A rash may be localized to one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, dry, cracked or blistered, swell and may be painful. An eruption in the skin which affects its appearance and/or texture. C36281 Integumentary System Finding C100104 Sign or Symptom C118468 Pediatric Rheumatology Terminology C116779 Spondylitis Spondylitis The inflammation of a vertebra. Inflammation of a vertebra. C2883 Arthritis C118468 Pediatric Rheumatology Terminology C116778 Spondyloarthritis Spondyloarthritis Spondyloarthropathy A group of inflammatory rheumatic diseases associated with arthritis and enthesitis, and often involving the axial skeleton. The most common form of spondyloarthritis is ankylosing spondylitis. Other forms include axial spondyloarthritis, peripheral spondyloarthritis, reactive arthritis, psoriatic arthritis/spondylitis and enteropathic arthritis/spondylitis. A group of inflammatory rheumatic diseases associated with arthritis and enthesitis, and often involving the axial skeleton. The most common form of spondyloarthritis is ankylosing spondylitis. Other forms include axial spondyloarthritis, peripheral spondyloarthritis, reactive arthritis, psoriatic arthritis/spondylitis and enteropathic arthritis/spondylitis. C2883 Arthritis C118468 Pediatric Rheumatology Terminology Seronegative Spondyloarthropathy C121368 Stress Dose Steroid Therapy Stress Dose Steroid The administration of corticosteroids to an individual with either a long term history of corticosteroid use, or a history of or increased risk for adrenal insufficiency during times of increased physiological stress, including surgery or serious illness. The administration of corticosteroids to an individual with either a long term history of corticosteroid use, or a history of or increased risk for adrenal insufficiency during times of increased physiological stress, including surgery or serious illness. C15986 Drug Therapy C118468 Pediatric Rheumatology Terminology C117111 Subacute Cutaneous Lupus Erythematosus Subacute Cutaneous Lupus Erythematosus A dermatologic manifestation of lupus involving erythematous, scaly patches or plaques, generally appearing on the upper back, chest, and arms, and often following sun exposure. It most often resolves without scarring. A dermatologic manifestation of lupus involving erythematous, scaly patches or plaques, generally appearing on the upper back, chest, and arms, and often following sun exposure. It most often resolves without scarring. C26819 Cutaneous Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C121650 Superficial Circumscribed Morphea Superficial Circumscribed Morphea Plaque Morphea Circumscribed morphea in which the lesions are restricted to the skin and the immediately underlying subcutaneous tissues. Circumscribed morphea in which the lesions are restricted to the skin and the immediately underlying subcutaneous tissues. C116784 Circumscribed Morphea C118468 Pediatric Rheumatology Terminology C119049 Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome PPHS|Pustulo-Psoriatic Hyperostotic Spondyloarthritis|SAPHO Syndrome A syndrome characterized by synovitis, acne, pustulosis, hyperostosis, and non-infectious osteomyelitis. Includes a spectrum of aseptic neutrophilic dermatoses associated with aseptic osteoarticular lesions. A syndrome characterized by synovitis, acne, pustulosis, hyperostosis, and non-infectious osteomyelitis. Includes a spectrum of aseptic neutrophilic dermatoses associated with aseptic osteoarticular lesions. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C122080 Systemic Corticosteroid Therapy Systemic Corticosteroid Treatment with corticosteroids via a delivery method that will affect the entire body (oral, intramuscular, intravenous). Treatment with corticosteroids via a delivery method that will affect the entire body (oral, intramuscular, intravenous). C15986 Drug Therapy C118468 Pediatric Rheumatology Terminology C119031 Systemic Juvenile Idiopathic Arthritis Systemic Juvenile Idiopathic Arthritis Systemic Onset Juvenile Idiopathic Arthritis|sJIA A category of juvenile idiopathic arthritis defined by the presence of arthritis and high fevers, and accompanied by at least 2 of the following systemic features: lymphadenopathy, organomegaly, rash, or serositis. Macrophage activation syndrome is a well known complication. A category of juvenile idiopathic arthritis defined by the presence of arthritis and high fevers, and accompanied by at least 2 of the following systemic features: lymphadenopathy, organomegaly, rash, or serositis. Macrophage activation syndrome is a well known complication. C114357 Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C61278 Still Disease Systemic Juvenile Rheumatoid Arthritis (AQ) An inflammatory disorder most often affecting children. It is characterized by the presence of arthritis, salmon-colored rash, spiking fevers, fatigue, and sore throats. An older, deprecated term for a category of juvenile rheumatoid arthritis that manifests with arthritis as well as systemic signs and symptoms, including high fevers, rash, hepatosplenomegaly, lymphadenopathy and anemia. C27179 Juvenile Rheumatoid Arthritis (AQ) C118468 Pediatric Rheumatology Terminology Still's Disease|Systemic JRA|Systemic Onset Juvenile Rheumatoid Arthritis|sJRA C3201 Systemic Lupus Erythematosus Systemic Lupus Erythematosus SLE An autoimmune multi-organ disease typically associated with vasculopathy and autoantibody production. Most patients have antinuclear antibodies (ANA). The presence of anti-dsDNA or anti-Smith antibodies are highly-specific. An autoimmune multi-organ disease typically associated with vasculopathy and autoantibody production. Most patients have antinuclear antibodies (ANA). The presence of anti-dsDNA or anti-Smith antibodies are highly-specific. C27153 Lupus Erythematosus C118468 Pediatric Rheumatology Terminology C97984 Systemic Lupus Erythematosus Disease Activity Index Systemic Lupus Erythematosus Disease Activity Index SLEDAI A clinical index for the measurement of disease activity in systemic lupus erythematosus. The index utilizes 24 weighted variables that measure disease activity, damage from disease, and health status. A global index that evaluates disease activity and includes twenty-four items collecting specific manifestations in nine organ systems: neurological, musculoskeletal, renal, mucocutaneous, general, heart, respiratory, vascular, and hematological. These items are scored based on whether these manifestations are present or absent in a specified time period. Several versions of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) are available. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121337 Systemic Lupus International Collaborating Clinics Classification Criteria Systemic Lupus International Collaborating Clinics Classification Criteria SLICC Criteria|Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus A validated revision of American College of Rheumatology (ACR) criteria for systemic lupus erythematosus with extension to seventeen criteria. Intended to be used as inclusion criteria for clinical studies, if the patient has biopsy-proven lupus nephritis with antinuclear antibodies (ANA) or anti-dsDNA, or the patient satisfies four of the criteria, including at least one clinical and one immunologic criterion. A validated revision of American College of Rheumatology (ACR) criteria for systemic lupus erythematosus with extension to seventeen criteria. Intended to be used as inclusion criteria for clinical studies, if the patient has biopsy-proven lupus nephritis with antinuclear antibodies (ANA) or anti-dsDNA, or the patient satisfies four of the criteria, including at least one clinical and one immunologic criterion. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C121353 Systemic Lupus International Collaborating Clinics/American College of Rheumatology-Damage Index Systemic Lupus International Collaborating Clinics/American College of Rheumatology-Damage Index SDI|SLICC/ACR Damage Index for SLE|SLICC/ACR-DI A clinical index used to measure accumulated organ damage from either the disease process or its sequelae, in twelve organ systems. The index scores damage regardless of cause. The definition of damage is an irreversible change in an organ or system that has occurred since the onset of systemic lupus erythematosus and is present for at least six months. A clinical index used to measure accumulated organ damage from either the disease process or its sequelae, in twelve organ systems. The index scores damage regardless of cause. The definition of damage is an irreversible change in an organ or system that has occurred since the onset of systemic lupus erythematosus and is present for at least six months. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C72070 Systemic Scleroderma Systemic Sclerosis Diffuse Scleroderma|SSc, Diffuse Sclerosis|Systemic Scleroderma A chronic disorder, possibly autoimmune, marked by excessive production of collagen which results in hardening and thickening of body tissues. The two types of systemic scleroderma, limited cutaneous and diffuse cutaneous are classified with focus on the extent of affected skin. A relationship exists between the extent of skin area affected and degree of internal organ/system involvement. Systemic scleroderma can manifest itself in pulmonary fibrosis, Raynaud's syndrome, digestive system telangiectasias, renal hypertension and/or pulmonary hypertension. A heterogeneous disorder characterized by small vessel vasculopathy, autoantibodies, and fibroblast dysfunction which results in hardening and thickening of body tissue. The clinical manifestations vary with the majority of the patients having skin thickening and involvement of internal organs. C26746 Scleroderma C118468 Pediatric Rheumatology Terminology C116789 Systemic Sclerosis without Skin Involvement Systemic Sclerosis without Skin Involvement SSC without Skin Involvement|Systemic Sclerosis sine Scleroderma A condition in which there are visceral manifestations of systemic sclerosis without any cutaneous findings. A condition in which there are visceral manifestations of systemic sclerosis without any cutaneous findings. C72070 Systemic Sclerosis C118468 Pediatric Rheumatology Terminology C35062 Takayasu Arteritis Takayasu Arteritis Pulseless Disease|Takayasu's Arteritis A large vessel vasculitis affecting the aorta and its branches. It usually affects young females. It causes vascular obstruction, resulting in asymmetric pulses. A large vessel granulomatous vasculitis affecting the aorta or its main branches and pulmonary arteries with angiographic findings of stenosis, occlusion, or aneurysm. Clinical findings include absent or asymmetric pulses (discrepancy in four limb blood pressure), bruits, hypertension, or elevation in acute phase reactants. C26912 Vasculitis C118468 Pediatric Rheumatology Terminology C122078 Topical Corticosteroid Therapy Topical Corticosteroid Treatment with a corticosteroid formulation designed for and administered onto a dermal, ocular or mucosal surface. Treatment with a corticosteroid formulation designed for and administered onto a dermal, ocular or mucosal surface. C121367 Localized Corticosteroid C118468 Pediatric Rheumatology Terminology C119051 Tumor Necrosis Factor Receptor 1-Associated Periodic Syndrome Tumor Necrosis Factor Receptor 1-Associated Periodic Syndrome Familial Hibernian Fever|TNF Receptor 1-Associated Periodic Syndrome|TRAPS An autoinflammatory disease caused by mutations in the TNFRSF1A gene coding for tumor necrosis factor receptor 1 (TNFR1). This results in attacks of fever, rash, peritoneal, pleural, or pericardial serositis and/or synovial inflammation along with increased acute phase reactants. Complications may include amyloidosis. An autoinflammatory disease caused by mutations in the TNFRSF1A gene coding for tumor necrosis factor receptor 1 (TNFR1). This results in attacks of fever, rash, peritoneal, pleural, or pericardial serositis and/or synovial inflammation along with increased acute phase reactants. Complications may include amyloidosis. C2889 Autoimmune Disease C3101 Genetic Disorder C118468 Pediatric Rheumatology Terminology C3426 Ulcer Ulcer Ulceration A circumscribed inflammatory and often suppurating lesion on the skin or an internal mucous surface resulting in necrosis of tissue. A circumscribed loss of integrity of the skin or mucous membrane. C36281 Integumentary System Finding C118468 Pediatric Rheumatology Terminology C116776 Undifferentiated Connective Tissue Disease Undifferentiated Connective Tissue Disease UCTD An autoimmune disorder which does not meet classification criteria used to establish the presence of other well-defined connective tissue diseases. An autoimmune disorder which does not meet classification criteria used to establish the presence of other well-defined connective tissue diseases. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology C119035 Undifferentiated Juvenile Idiopathic Arthritis Undifferentiated Juvenile Idiopathic Arthritis Undifferentiated JIA A category of juvenile idiopathic arthritis that does not fulfill any single category or has criteria for more than one category. A category of juvenile idiopathic arthritis that does not fulfill any single category or has criteria for more than one category. C114357 Juvenile Idiopathic Arthritis C118468 Pediatric Rheumatology Terminology C38095 Urine Casts Urinary Casts Urine Casts Tubules in the kidneys secrete proteins. Under some circumstances, these proteins precipitate out to form little cylindrical impressions of the tubules called casts. If anything is present in the tubules at the time, it gets trapped in the protein casts. This offers a "snap shot" of the tubular lumen at the time of cast formation. (from Medline Plus) Cylindrical impressions of kidney tubules and collecting ducts that comprise primarily of mucoprotein precipitates that can be overlaid with acellular (hyaline, granular, waxy, pigment, fat, crystals) or cellular (red blood cells, white blood cells, bacteria, or epithelial cells) components. C36286 Urinary System Finding C118468 Pediatric Rheumatology Terminology C26909 Uveitis Uveitis An inflammatory process affecting a part of or the entire uvea. Causes include inflammatory agents (e.g., herpes simplex, herpes zoster, leptospirosis) and systemic diseases (e.g., inflammatory bowel disease, multiple sclerosis, systemic lupus erythematosus, ankylosing spondylitis). Patients present with pain and redness in the eye, light sensitivity, and blurred and decreased vision. Inflammation of the iris, ciliary body, and/or choroid (uveal tract). C26767 Eye Disorder C118468 Pediatric Rheumatology Terminology C121364 Valentini Scleroderma Disease Activity Index Valentini Scleroderma Disease Activity Index Valentini SDAI A clinical index used to assess the activity of scleroderma that has been validated in adults. A clinical index used to assess the activity of scleroderma that has been validated in adults. C20993 Clinical Assessment Tool C118468 Pediatric Rheumatology Terminology C119997 Vasculitic Ulcer Vasculitic Ulcer A circumscribed inflammatory lesion associated with underlying ischemic disease. A circumscribed inflammatory lesion associated with underlying ischemic disease. C3426 Ulcer C118468 Pediatric Rheumatology Terminology C26912 Vasculitis Vasculitis An inflammatory process involving the wall of the vessels (large, medium, or small-sized vessels). The inflammatory process may cause necrosis or formation of granulomas in the vascular wall. It may be the result of an autoimmune disorder, infection, or malignancy. Representative examples include polyarteritis nodosa, temporal arteritis, Wegener granulomatosis, Kawasaki disease, Takayasu arteritis, and Henoch-Schonlein purpura. An inflammatory process involving the wall of either arteries or veins, often classified by vessel size (large, medium, or small). The characteristic histopathology reveals inflammatory cells within the vessel wall accompanied by fibrinoid necrosis. Vasculitis may be the result of an autoimmune disorder, infection, or malignancy. C2889 Autoimmune Disease C118468 Pediatric Rheumatology Terminology