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Adult Still's disease: manifestations, disease course, and outcome in 62

Pouchot J, Sampalis JS, Beaudet F, Carette S, Decary F,
Salusinsky-Sternbach M, Hill RO, Gutkowski A, Harth M, Myhal D, et al
Service de Medecine Interne, Hopital Louis Mourier, Colombes, France.

Clinical and laboratory manifestations, disease course, outcome, and HLA
associations were studied in an inception cohort of 62 subjects with
adult Still's disease (ASD) from 5 Canadian universities. 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
pain (48%) were noted in our patients compared to those in a recent
literature review. 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 therapy.
Therefore, whether or not aspirin or other NSAIDs are used, we recommend
close monitoring of LFTs in patients with ASD, especially early in the
disease course. Laboratory manifestations were similar to those already
reported. Leukocytosis (greater than or equal to 15,000/mm3) was present
in 50 patients (81%), a normochromic, normocytic anemia (hemoglobin less
than or equal to 10 g/dl) in 42 (68%), and an elevated ESR in all. 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.

PMID: 2005777, UI: 91171847



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