| Entry |
|
|---|
| Name |
Juvenile idiopathic arthritis |
|---|
| Subgroup |
Juvenile rheumatoid arthritis
Still's disease
Juvenile arthritis (JUVAR) |
|---|
| Description |
Juvenile idiopathic arthritis (JIA) is one of the most common childhood rheumatic diseases. Clinically, it is defined as arthritis of unknown origin that starts before the age of 16, and persists for at least 6 weeks. Next to a certain genetic predisposition, environmental factors play a role leading to a chronic inflammatory response. JIA is not a single disorder but consists of a heterogeneous group of auto-immune inflammatory diseases. It has variable rates in course and activity of disease. Based on 6 months of clinical symptoms and global prognostic factors, the following clinical subtypes of JIA are recognized: systemic JIA, oligoarthritis, RF-negative polyarthritis, RF-positive polyarthritis, psoriatic JIA, enthesitis- related arthritis, and undifferentiated arthritis. Systemic JIA, also known as Still's disease, is a subtype with strong systemic clinical symptoms. Patients with systemic JIA have, in addition to arthritis, prominent symptoms of systemic inflammation such as spiking fever, rash, pericarditis, peritonitis, lymphadenopathy and organomegaly. A severe and often life-threatening complication occurring in 10-30% of patients with systemic JIA is macrophage activation syndrome. Polymorphisms in the IL6 and in the MIF gene have been found to be associated with susceptibility to the disorder. Based on the known relevance of IL6 in JIA pathophysiology, tocilizumab has been investigated and approved for use in the treatment of systemic and polyarticular JIA.
|
|---|
| Category |
Immune system disease
|
|---|
| Brite |
Human diseases [BR:br08402]
Immune system diseases
Allergies and autoimmune diseases
H01672 Juvenile idiopathic arthritis
Human diseases in ICD-11 classification [BR:br08403]
15 Diseases of the musculoskeletal system or connective tissue
Arthropathies
Inflammatory arthropathies
FA24 Juvenile idiopathic arthritis
H01672 Juvenile idiopathic arthritis
|
|---|
| Pathway |
| hsa04060 Cytokine-cytokine receptor interaction | |
|
|---|
| Gene |
|
|---|
| Drug |
|
|---|
| Comment |
See also H01516 Adult onset Still's disease.
|
|---|
| Other DBs |
|
|---|
| Reference |
|
|---|
| Authors |
Swart JF, de Roock S, Prakken BJ |
|---|
| Title |
Understanding inflammation in juvenile idiopathic arthritis: How immune biomarkers guide clinical strategies in the systemic onset subtype. |
|---|
| Journal |
|
|---|
| Reference |
|
|---|
| Authors |
Vivarelli M, D'Urbano LE, Insalaco A, Lunt M, Jury F, Tozzi AE, Ravelli A, Martini A, Donn R, De Benedetti F |
|---|
| Title |
Macrophage migration inhibitory factor (MIF) and oligoarticular juvenile idiopathic arthritis (o-JIA): association of MIF promoter polymorphisms with response to intra-articular glucocorticoids. |
|---|
| Journal |
Clin Exp Rheumatol 25:775-81 (2007) |
|---|
| Reference |
|
|---|
| Authors |
Woo P, Humphries SE |
|---|
| Title |
IL-6 polymorphisms: a useful genetic tool for inflammation research? |
|---|
| Journal |
|
|---|
| Reference |
|
|---|
| Authors |
Kallinich T, Thorwarth A, von Stuckrad SL, Rosen-Wolff A, Luksch H, Hundsdoerfer P, Minden K, Krawitz P |
|---|
| Title |
Juvenile arthritis caused by a novel FAMIN (LACC1) mutation in two children with systemic and extended oligoarticular course. |
|---|
| Journal |
|
|---|
| LinkDB |
|
|---|
