Juvenile idiopathic arthritis causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Dheeraj Makkar, M.D.[2] Nehal Eid, M.D.[3]
Overview
Juvenile idiopathic arthritis (JIA) is a multifactorial inflammatory disease with no single identifiable cause. It arises from the interaction of genetic susceptibility, immune dysregulation, and environmental triggers. The relative contribution of autoimmune and autoinflammatory mechanisms varies by subtype, with adaptive immunity predominating in most forms and innate immune activation central to systemic JIA.
Causes
The exact cause of juvenile idiopathic arthritis (JIA) is unknown. JIA is considered a multifactorial disease resulting from the interaction of genetic susceptibility, immune dysregulation, and environmental triggers.
- Genetic factors: Certain genetic variants, including associations with human leukocyte antigen (HLA) alleles, increase susceptibility to JIA. Genetic risk differs among subtypes, with stronger autoimmune associations in oligoarticular and polyarticular JIA and innate immune pathway involvement in systemic JIA.
- HLA-DRB1*11 is present in systemic JIA.
- HLA-DRB1*15 is associated with lung disease. *
- HLA-B27 is present in many ERA patients.
- Variants in HLH-related genes have been found in up to one third of patients with systemic JIA in whom macrophage activation syndrome devel oped.56,57[1][2]
- Immune dysregulation: Abnormal activation of the immune system leads to persistent synovial inflammation. Adaptive immune mechanisms predominate in most JIA subtypes, whereas systemic JIA is driven primarily by innate immune activation.
- Environmental triggers: Infections or other environmental exposures may act as initiating triggers in genetically predisposed individuals, although no single infectious agent has been consistently identified.
- Loss of immune tolerance: Failure of normal immune regulatory mechanisms results in sustained inflammation, autoantibody production in some subtypes, and chronic joint damage.
Overall, JIA arises from a complex interplay of genetic and environmental factors leading to chronic immune-mediated inflammation rather than a single identifiable cause.
The cause of JIA, as the word idiopathic suggests, was supposed to be unknown and currently an area of active research.[4] Current understanding of JIA also suggests that it arises in a genetically susceptible individual due to environmental factors.[5]
References
- ↑ Kaufman KM, Linghu B, Szustakowski JD, et al. Whole-exome sequencing reveals overlap between macrophage activation syndrome in systemic juvenile idiopathic arthritis and familial hemophagocytic lymphohistiocytosis. Arthritis Rheumatol 2014; 66: 3486-95.
- ↑ Correia Marques M, Rubin D, Shuld iner EG, et al. Enrichment of rare variants of hemophagocytic lymphohistiocytosis genes in systemic juvenile idiopathic arthritis. Arthritis Rheumatol 2024; 76: 1566-72.
- ↑ Sandborg CI, Schulert GS, Kimura Y. Juvenile Idiopathic Arthritis. N Engl J Med. 2025;392:XXX–XXX. doi:10.1056/NEJMra2402073.
- ↑ Phelan J, Thompson S (2006). "Genomic progress in pediatric arthritis: recent work and future goals". Curr Opin Rheumatol. 18 (5): 482–9. PMID 16896287.
- ↑ Førre O, Smerdel A (2002). "Genetic epidemiology of juvenile idiopathic arthritis". Scand J Rheumatol. 31 (3): 123–8. PMID 12195624.