C 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 C 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 C 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 C C27179 Juvenile Rheumatoid Arthritis (AQ) JRA 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 The juvenile rheumatoid arthritis classification system has been replaced by the International League of Associations for Rheumatology (ILAR) juvenile idiopathic arthritis classification system.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 The juvenile rheumatoid arthritis classification system has been replaced by the International League of Associations for Rheumatology (ILAR) juvenile idiopathic arthritis classification system.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 C 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 C C50464 Arthralgia Arthralgia Joint Pain Pain in a joint. Pain in a joint. C3303 Pain C100104 Sign or Symptom C118468 Pediatric Rheumatology Terminology C 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 C 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 C 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 C 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 C 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 C 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 C 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 C 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 C 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 C 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 C 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 C 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 C 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 C C34831 Myocarditis Myocarditis Inflammation of the muscle tissue of the heart. Inflammation of the myocardium. C3079 Heart Disorder C118468 Pediatric Rheumatology Terminology C 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 C 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 C 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 C 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 C 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 C C3090 Hematuria Hematuria Blood in the urine. Blood in the urine. C3149 Kidney Disease C118468 Pediatric Rheumatology Terminology C 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 C 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 C 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 C 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 C 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 C 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 C C35109 Anterior Uveitis Anterior Uveitis Iridocyclitis|Iritis Inflammation of the iris and anterior chamber of the eye. Inflammation of the anterior uveal tract. C26909 Uveitis C118468 Pediatric Rheumatology Terminology C 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 C 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 D C29505 Topical Corticosteroid Topical Corticosteroid Any synthetic steroid derivative exhibiting the same function as the naturally occurring corticosteroid hormone, formulated for topical application. Topical corticosteroids are applied to the skin where it exerts its effect, however, corticosteroids can be absorbed systemically after being applied locally. Topical corticosteroids are mainly used for the localized treatment of inflammation of the skin and help relieve symptoms such as itching, swelling and redness. A corticosteroid formulation designed for and administered onto a dermal, ocular or mucosal surface. C2322 Corticosteroid C118468 Pediatric Rheumatology Terminology D C80510 Steroid, Systemic Systemic Corticosteroid Systemic Steroid Synthetically made hormones possessing the steroid ring system; e.g., androgens, estrogens, and adrenocortical hormones. This does not include topical or inhaled steroid therapy. Systemically-delivered (oral, intramuscular, intravenous) corticosteroids. C2322 Corticosteroid C118468 Pediatric Rheumatology Terminology