Adult-onset Still disease in southeast Brazil.
BACKGROUND
Adult-onset Still disease (AOSD) has been described all over the world. Clinical presentations and prognosis have varied in different studies.


OBJECTIVE
The objective of this study was to determine the clinical presentation and the evolution of AOSD at a tertiary referral center in southeast Brazil.


METHODS
The clinical records of 16 patients were retrospectively studied to determine symptoms at diagnosis, follow up, and the medication prescribed.


RESULTS
The mean age at onset was 30.8 years (range, 24-55 years; standard deviation [SD], 9.2 years) with a slight male prevalence (54.2%). All patients presented constitutional symptoms, fever, and skin rash. Liver involvement was observed in all cases, with hepatomegaly in 81.3%, increased liver enzymes in 50.0%, and hypergammaglobulinemia in 68.8%. Cardiac involvement was observed in 12.6%, pleuritis in 6.3%, and renal involvement in 25.0%. All patients presented leukocytosis with a predominance of neutrophils. Elevated ferritin levels were observed in 56.3%, and these levels were normalized after disease remission. Initial treatments included nonsteroidal antiinflammatory drugs and low-dosage corticosteroids in all patients; 43.8% also needed methotrexate. In 25.0% of cases, a monocyclic disease was observed; others had recurrent episodes. After a follow up of 6.9 years (SD, 1.2 years), carpal ankylosis was the main articular sequel, observed in 53.6% of the patients.


CONCLUSIONS
AOSD is rare in southeast Brazil. Although less severe systemic manifestations, like serositis and pneumonitis, were observed, reversible liver involvement was common; the frequency of recurrent disease and carpal ankylosis was higher than in previous studies.
Appenzeller
Simone
S
Department of Internal Medicine, State University of Campinas (UNICAMP), CEP 13081-970 Campinas, São Paulo, Brazil. appenzel@unicamp.br
Castro
Glaucio R W
GR
Costallat
Lilian T L
LT
Samara
Adil M
AM
Bértolo
Manoel B
MB
D000328:Adult; D001175:Arthrography; D001772:Blood Cell Count; D001799:Blood Sedimentation; D001938:Brazil; D005260:Female; D005293:Ferritins / Q000097:blood; D005500:Follow-Up Studies; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007677:Kidney Function Tests; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016706:Still's Disease, Adult-Onset / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging*; D016896:Treatment Outcome
D006454:Hemoglobins; D005293:Ferritins