Adult-Onset Still's Disease
Adult-onset Still's disease (AOSD) was described originally by Eric Bywaters in 1971. AOSD closely resembles systemic-onset juvenile idio-pathic arthritis, a pediatric disorder known originally as Still's disease. An intensely inflammatory, multiorgan system disorder characterized by a polyarthritis, high spiking fevers, and an evanescent, salmon-colored rash (Figs. 3.1a–c). The rash is described as small macules that disappear during the night but reappear the next day, usually coincident with a fever spike. Peripheral joint involvement can be fleeting but also may settle into a refractory, destructive arthritis marked by a tendency in some joints for fusion. AOSD is typically seronegative. Patients do not have rheumatoid factor or antibodies to cyclic citrullinated peptides. Other common features are leukocytosis, thrombocy-tosis, elevations of the serum hepatic aminotransferase concentrations, splenomegaly, and serositis. Pharyngitis is often the initial symptom.
KeywordsJuvenile Idiopathic Arthritis Interstitial Lung Disease Familial Mediterranean Fever Juvenile Rheumatoid Arthritis Serum Ferritin Level
- Bresnihan B. Cytokine neutralizers: IL-1 inhibitors. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (eds) Rheumatology (4th edn). Edinburgh, UK, Mosby, 2008, pp. 495–500Google Scholar
- Esdaile J. Adult Still's disease. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (eds) Rheumatology (4th edn). Edinburgh, UK, Mosby, 2008, pp. 785–792Google Scholar