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

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.