Adult Onset Still's Disease: Clinical Features and Prognostic Factors in Korea

Cho, Kyoon Seok; Yoo, Dae Hyun; Yun, Hyung Ran; Lee, Myong Ho; Lee, Je Kyung; Shim, Seung Cheol; Chang, Dae Kook; Yoo, Tae Seok; Koh, Hee Kwan; Kim, Tae Hwan; Jun, Jae Bum

Abstract

OBJECTIVE
This study was undertaken to review the disease course, clinical and laboratory manifestations, prognosis and treatment of adult onset Still s disease (AOSD) in Korea. METHODS: Thirty-two patients with AOSD were enrolled from 1986 to 1997 in Hanyang University Hospital. Diagnosis of AOSD was based on the criteria proposed by Yamaguchi. We classified the disease course into self-limited, inter mittent, or chronic disease course. RESULTS: Twenty-four (75%) patients were female. Skin rash occurred in 28 (88%) patients, lymphadenopathy in 8 (25%), hepatomegaly in 4 (13%), and pericarditis in 2 (6%) out of 32 patients. The most commonly affected joints were knee joints (88%). Elevated LDH was seen in 18 (60%) patients and decreased CK in 17 (61%) patients. Rheumatoid factor was detected in 4 (13%) patients and ANA in 12 (38%) patients. Anemia (Hb < 10 g/dL) was seen in 13 (41%) patients and hypoalbuminemia (<3. 5 g/dL) in 14 (52%) patients. Elevated ferritin (300 ng/mL) level was seen in 23 (79%) patients. Twenty-five (78%) patients had elevated serum transaminase. Bone marrow studies were performed in 16 patients. Nine out of 16 patients showed hyperplasia of the myeloid series and 2 patients displayed the features of a hemophagocytic syndrome. The mean duration of follow up of 32 patients was 32 months (range 3- 108). Eight (27%) patients had a self-limited, 9 (30%) an intermittent, and 13 (43%) a chronic disease course. The hypoalbuminemia was significantly associated with an "intermittent or chronic disease group" (p<0. 05). Thirty-two patients received systemic corticosteroids and 21 patients received single or combination of disease modifying antirheumatic drugs. CONCLUSION: We found that hypoalbuminemia at presentation was significantly associated with an unfavorable outcome, intermittent or chronic disease group. The clinical manifestations and disease course of AOSD in Korea were similar to those previously reported in other countries except significantly lower incidence of lymphadenopathy, hepatomegaly, and pericarditis.