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Adult Still's disease: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease. It is characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash. The Levels of the iron-binding protein ferritin may be elevated with this disorder. AOSD may present in a similar manner to other inflammatory diseases and to autoimmune diseases The cause of adult-onset Still's disease is not known, but it presumably involves interleukin-1 (IL-1), since drugs that block the action of IL-1β are effective in treatment. Interleukin-18 is expressed at high levels
This disease presents with joint pain, high fevers, a salmon-pink rash, enlargement of the liver and spleen, swollen lymph nodes, and an increased white blood cell count in the blood. Tests for rheumatoid factor and anti-nuclear antibodies are usually negative and serum ferritin is elevated. Patients experience extreme fatigue, swelling of the lymph nodes, and less commonly fluid accumulation in the lungs and heart. In rare cases, AOSD can cause aseptic meningitis and sensorineural hearing loss. There are two types of criteria- minor and major criteria, In which at least two of these being major diagnostic criteria.
Treatment: Patients with disease are divided into monocyclic systemic disease, polycyclic systemic disease, chronic articular monocyclic systemic disease, and chronic articular polycyclic systemic disease. Chronic articular disease and polyarticular disease were at higher risk to develop disabling arthritis. It is treated with anti-inflammatory drugs. Other commonly used medications include hydroxychloroquine, penicillamine, azathioprine, methotrexate, etanercept, anakinra, cyclophosphamide, adalimumab, rituximab, and infliximab. Newer drugs target interleukin-1 (IL-1), particularly IL-1β. A randomized, multicenter trial reported better outcomes in a group of 12 patients treated with anakinra than in a group of 10 patients taking other disease-modifying antirheumatic drugs. Other anti-IL1β drugs are being developed, including canakinumab and rilonacept.
Statistics: The approximate Adult Stills Disease incidence in Central Europe, Switzerland was found to be 1,013,537. Adult-onset Still's Disease is rare and has been described all over the world. The number of new cases of AOSD per year is estimated to be 0.16 new cases per 100,000 populations. Prevalence is estimated at 1.5 cases per 100,000-1,000,000 population