Adult-Onset Still Disease in Southeast Brazil

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Adult-Onset Still Disease in Southeast Brazil.

JCR: Journal of Clinical Rheumatology. 11(2):76-80, April 2005.

Appenzeller, Simone MD; Castro, Glaucio R.W. MD; Costallat, Lilian T.L. MD,

PhD; Samara, Adil M. MD, PhD; Bertolo, Manoel B. MD, PhD

http://www.mdlinx.com/RheumatologyLinx/theaarts.cfm?artid=1203860 & specid=18 & ok=y\

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Abstract:

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.

Conclusion: 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.

© 2005 Lippincott & Wilkins, Inc.