Adult-Onset Still's Disease

Abstract

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.