Adult Still's disease:
manifestations, disease course, and outcome in 62
Twenty-eight patients (45%)
were female and the median age at disease onset was 24 years. In general, the
clinical features observed in our patients were identical to those in other
published series. However, significantly higher frequencies of sore throat
(92%), weight loss (76%), lymphadenopathy (74%), pleuritis (53%), pneumonitis
(27%), and abdominal
Liver involvement with
hepatomegaly (44%) or abnormal liver function tests (LFTs) (76%) was common and
was responsible for the 2 deaths attributed to Still's disease in our series.
Severe liver failure always occurred in conjunction with aspirin or NSAID
Laboratory manifestations were
similar to those already reported. Leukocytosis (greater than or equal to
15,000/mm3) was present
The mean follow-up of the 62 patients was 70 months (range, 2-163). Twenty-one patients (34%) had a self-limited disease course, 15 (24%) an intermittent course, and 22 (36%) a chronic disease course. Four patients (6%) died, and 2 of these deaths were attributed to Still's disease. For those patients who experienced a recurrence of ASD, the flares were usually of shorter duration and milder in severity than the initial episode.
No initiating factor for disease exacerbation was identified in our patients. Although 22 of 62 patients (36%) had a chronic disease course, 52 (90%) were in ARA Functional Class I, and only 4 and 2 patients were in ARA Functional Class II and III, respectively. Patients with Still's disease had higher scores than the controls on the Pain (P less than 0.01) and Physical Disability (P less than 0.05) subscales of Arthritis Impact Measurement Scales health status questionnaire.
Joint radiographs performed at the follow-up evaluation disclosed typical carpometacarpal and intercarpal involvement in 16 of 39 patients. In our series, HLA-B17, B18, B35, and DR2 were significantly associated with ASD. Three significant predictors of an unfavorable outcome, either a chronic disease course or a longer time to clinical remission, were identified.
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