| H01672 | |
| H number | H01672 |
| Name | Juvenile idiopathic arthritis |
| 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 |
| Network | - |
| Gene | IL6 [HSA:3569] [KO:K05405] MIF [HSA:4282] [KO:K07253] (JUVAR) LACC1 [HSA:144811] [KO:K05810] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| 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] 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] |
| Comment | See also H01516 Adult onset Still's disease. |
| Other DBs | ICD-11: FA24 ICD-10: M08 MeSH: D001171 OMIM: 604302 618795 |
| Reference | PMID:27461267 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 PMID:9769329 (IL6) AUTHORS Fishman D, Faulds G, Jeffery R, Mohamed-Ali V, Yudkin JS, Humphries S, Woo P TITLE 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. JOURNAL J Clin Invest 102:1369-76 (1998) DOI:10.1172/JCI2629 PMID:11508429 (MIF) AUTHORS Donn RP, Shelley E, Ollier WE, Thomson W TITLE A novel 5'-flanking region polymorphism of macrophage migration inhibitory factor is associated with systemic-onset juvenile idiopathic arthritis. JOURNAL Arthritis Rheum 44:1782-5 (2001) DOI:10.1002/1529-0131(200108)44:8<1782::AID-ART314>3.0.CO;2-# PMID:27881174 (LACC1) 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 Pediatr Rheumatol Online J 14:63 (2016) DOI:10.1186/s12969-016-0124-2 |