KEGG   DISEASE: Juvenile idiopathic arthritisHelp
H01672                      Disease                                

Juvenile idiopathic arthritis
Juvenile rheumatoid arthritis
Still's disease
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.
Immune system disease
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
   Inflammatory arthropathies
    FA24  Juvenile idiopathic arthritis
     H01672  Juvenile idiopathic arthritis
BRITE hierarchy
hsa04060 Cytokine-cytokine receptor interaction   
hsa04630 Jak-STAT signaling pathway   
IL6 (polymorphism) [HSA:3569] [KO:K05405]
MIF (polymorphism) [HSA:4282] [KO:K07253]
Triamcinolone acetonide [DR:D00983]
Dexamethasone [DR:D00292]
Dexamethasone sodium phosphate [DR:D00975]
Hydrocortisone [DR:D00088]
Hydrocortisone sodium succinate [DR:D00978]
Prednisolone [DR:D00472]
Prednisolone sodium phosphate [DR:D00981]
Prednisone [DR:D00473]
Sulfasalazine [DR:D00448]
Methylprednisolone [DR:D00407]
Methylprednisolone sodium succinate [DR:D00751]
Methylprednisolone acetate [DR:D00979]
Corticotropin [DR:D00146]
Cortisone acetate [DR:D00973]
Methotrexate [DR:D00142]
Methotrexate sodium [DR:D02115]
Abatacept [DR:D03203]
Etanercept [DR:D00742]
Adalimumab [DR:D02597]
Tocilizumab [DR:D02596]
Canakinumab [DR:D09315]
Oxaprozin [DR:D00463]
Naproxen and esomeprazole magnesium [DR:D11576]
See also H01516 Adult onset Still's disease.
Other DBs
ICD-11: FA24
ICD-10: M08
MeSH: D001171
OMIM: 604302
PMID:27461267 (description, marker)
Swart JF, de Roock S, Prakken BJ
Understanding inflammation in juvenile idiopathic arthritis: How immune biomarkers guide clinical strategies in the systemic onset subtype.
Eur J Immunol 46:2068-77 (2016)
Vivarelli M, D'Urbano LE, Insalaco A, Lunt M, Jury F, Tozzi AE, Ravelli A, Martini A, Donn R, De Benedetti F
Macrophage migration inhibitory factor (MIF) and oligoarticular juvenile idiopathic arthritis (o-JIA): association of MIF promoter polymorphisms with response to intra-articular glucocorticoids.
Clin Exp Rheumatol 25:775-81 (2007)
Woo P, Humphries SE
IL-6 polymorphisms: a useful genetic tool for inflammation research?
J Clin Invest 123:1413-4 (2013)
PMID:26848760 (drug)
Turnier JL, Brunner HI
Tocilizumab for treating juvenile idiopathic arthritis.
Expert Opin Biol Ther 16:559-66 (2016)
PMID:26678914 (drug)
Hugle B, Horneff G
The role of synthetic drugs in the biologic era: therapeutic strategies for treating juvenile idiopathic arthritis.
Expert Opin Pharmacother 17:703-14 (2016)
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