Clinical manifestations, disease course, and complications of adult-onset Still's disease in Taiwan

Abstract

Background and purpose: Data on the disease course and ultimate outcome of adult-onset Still's disease (AOSD) are limited. We analyzed the clinical manifestations, disease course, and complications of patients with AOSD in Taiwan.

Methods: A retrospective cohort design with prospective follow-up was used. Eighty two patients with AOSD diagnosed between 1983 and 2003 were evaluated. Their clinical features and laboratory findings at presentation, disease course, and complications were analyzed.

Results: Fifty nine patients (72%) were female and 55 (67.1%) were aged between 16-35 years at onset. The most common clinical manifestations were fever (100%), articular symptoms (100%), evanescent rash (87%), and sore throat (84%). Dermatographism was noted in 59% of patients. Elevation of erythrocyte sedimentation rate and C-reactive protein, which were significantly correlated with disease activity score (both p < 0.01) occurred in more than 90% of AOSD patients. Elevation of serum ferritin, which was significantly correlated with disease activity score and hepatic enzyme levels, was present in 91% of patients. Polycyclic systemic course was the most common (45%), followed by monocyclic systemic course (34%); only 20% of patients progressed to chronic arthropathy.

Conclusions: The multisystemic involvement and various patterns of disease course in this series illustrate the heterogenic nature of AOSD. Serum ferritin levels can be used as a marker for monitoring disease activity in AOSD.