KEGG   DISEASE: Juvenile idiopathic arthritis
H01672                      Disease                                
Juvenile idiopathic arthritis
Juvenile rheumatoid arthritis
Still's disease
Juvenile arthritis (JUVAR)
Rheumatoid arthritis [DS:H00630]
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
hsa05323  Rheumatoid arthritis
hsa04060  Cytokine-cytokine receptor interaction
hsa04630  Jak-STAT signaling pathway
IL6 [HSA:3569] [KO:K05405]
MIF [HSA:4282] [KO:K07253]
(JUVAR) LACC1 [HSA:144811] [KO:K05810]
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]
Naproxen [DR:D00118]
Naproxen sodium [DR:D00970]
Corticotropin [DR:D00146]
Cortisone acetate [DR:D00973]
Methotrexate [DR:D00142]
Methotrexate sodium [DR:D02115]
Celecoxib [DR:D00567]
Abatacept [DR:D03203]
Tofacitinib citrate [DR:D09783]
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 618795
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)
PMID:9769329 (IL6)
Fishman D, Faulds G, Jeffery R, Mohamed-Ali V, Yudkin JS, Humphries S, Woo P
The effect of novel polymorphisms in the interleukin-6 (IL-6) gene on IL-6 transcription and plasma IL-6 levels, and an association with systemic-onset juvenile chronic arthritis.
J Clin Invest 102:1369-76 (1998)
PMID:11508429 (MIF)
Donn RP, Shelley E, Ollier WE, Thomson W
A novel 5'-flanking region polymorphism of macrophage migration inhibitory factor is associated with systemic-onset juvenile idiopathic arthritis.
PMID:27881174 (LACC1)
Kallinich T, Thorwarth A, von Stuckrad SL, Rosen-Wolff A, Luksch H, Hundsdoerfer P, Minden K, Krawitz P
Juvenile arthritis caused by a novel FAMIN (LACC1) mutation in two children with systemic and extended oligoarticular course.
Pediatr Rheumatol Online J 14:63 (2016)

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