KEGG   DISEASE: Juvenile idiopathic arthritisHelp
Entry
H01672                      Disease                                

Name
Juvenile idiopathic arthritis
  Subgroup
Juvenile rheumatoid arthritis
Still's disease
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
BRITE hierarchy
Pathway
hsa04060 Cytokine-cytokine receptor interaction   
hsa04630 Jak-STAT signaling pathway   
Gene
IL6 (polymorphism) [HSA:3569] [KO:K05405]
MIF (polymorphism) [HSA:4282] [KO:K07253]
Drug
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]
Comment
See also H01516 Adult onset Still's disease.
Other DBs
ICD-11: FA24
ICD-10: M08
MeSH: D001171
OMIM: 604302
Reference
PMID:27461267 (description, marker)
  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
Eur J Immunol 46:2068-77 (2016)
DOI:10.1002/eji.201546092
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
J Clin Invest 123:1413-4 (2013)
DOI:10.1172/JCI67221
Reference
PMID:26848760 (drug)
  Authors
Turnier JL, Brunner HI
  Title
Tocilizumab for treating juvenile idiopathic arthritis.
  Journal
Expert Opin Biol Ther 16:559-66 (2016)
DOI:10.1517/14712598.2016.1150997
Reference
PMID:26678914 (drug)
  Authors
Hugle B, Horneff G
  Title
The role of synthetic drugs in the biologic era: therapeutic strategies for treating juvenile idiopathic arthritis.
  Journal
Expert Opin Pharmacother 17:703-14 (2016)
DOI:10.1517/14656566.2016.1133592
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