Adult-onset Still's disease overview
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Adult-onset Still's disease |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]
Overview
Historical Perspective
Adult-onset Still's disease (AOSD) is an inflammatory condition characterized by high spiking fever, rash, sore throat, and joint pain. In 1896, an English doctor named George Frederick Still, described the condition in children and the disease is named after him. In 1971, EG Bywaters described the term "adult Still's disease" which was later used for adults who had a condition similar to systemic onset JRA. There's no cure for adult-onset Still's disease; however, symptomatic treatment using corticosteroids, anti-interleukinagents and disease modifying anti-rheumatic drugs (DMARDs) may provide relief, and aid in remission.
Classification
Adult-onset Stiil's disease (AOSD) may be classified based on the predominant clinical picture with which the patient presents into systemic sub-type and chronic arthritis sub-type. The sub-types differ based on the cytokine profile, clinical course and response to treatment.
Pathophysiology
Adult-onset Still's disease (AOSD) is an autoimmune inflammatory arthritis that typically affects adolescents and adults ranging from age 16-40 years. Major etiological mechanisms behind cause a dysfunction of the innate and cellular immunity (limited) leading to activation of effector cells of the disease. Although the pathogenesis of adult-onset Still's disease is largely knwon to be idiopathic. Triggers of AOSD lead to activation of toll-like receptors (TLR) and activation of immune system. Pathogen-associated molecular patterns (PAMPs) and danger-associated molecular patterns (DAMPs) play an important role in the etiopathogenesis of AOSD. They lead to release of various cytokines in the body such as interleukin-1 beta (IL-1), interleukin-6 (IL-6), interleukin-17, interleukin-18, interferon-alpha (IFN-alpha) and tumor necrosis factor (TNF-alpha) . These cytokines play major roles in modifying the normal working of the body and produce the typical clinical pictiure associated with AOSD. Some distinct HLA alleles have been shown to be associated with AOSD such as HLA-DR4, HLA-Bw35 (associated with good prognosis), HLA-DRB1, HLA-DRw6 (joint root involvement), HLA-B17, HLA-B35 and HLA-DR2. On gross examination, the involved joints may exhibit soft tissue swelling, cartilage loss, jointerosions and carpal ankylosis (late during the disease process). On microscopic examination of the joint fluid, typical features associated with inflammatory joint disease may be observed.