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Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology that is responsible for a significant proportion of cases of fever of unknown origin and can also have serious musculoskeletal sequelae.To assess and synthesise the evidence for optimal diagnosis and management of AOSD.The key terms, adult onset Still’s disease, AOSD, adult Still’s disease, ASD, Still’s disease were used to search Medline (1966–2005) and PubMed (1966–2005) for all available articles in the English language. Clinically relevant articles were subsequently selected. Bibliographies, textbooks, and websites of recent rheumatology conferences were also assessed.Data on diagnosis and treatment of AOSD are limited in the medical literature and consist mainly of case reports, small series, and modest scale retrospective studies. Diagnosis is clinical and requires exclusion of infectious, neoplastic, and other autoimmune diseases. Laboratory tests are non-specific and reflect heightened immunological activity. Treatment comprises non-steroidal anti-inflammatory drugs, corticosteroids, immunosuppressive drugs (methotrexate, leflunomide, gold, azathioprine, ciclosporin A, cyclophosphamide), and intravenous gammaglobulin. The recent successful application of biological agents (anti-tumour necrosis factor, anti-interleukin (IL)1, anti-IL6), often in combination with traditional immunosuppressive drugs, has been very promising.AOSD often poses a diagnostic and therapeutic challenge and clinical guidelines are lacking. The emergence of validated diagnostic criteria, discovery of better serological markers, and the application of new biological agents may all provide the clinician with significant tools for the diagnosis and management of this complex systemic disorder.