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Adult-onset still disease: diagnosis and evolution

OBJECTIVE: To determine the clinical manifestations at disease onset and during follow-up of patients with Adult-Onset Still Disease (AOSD) in a tertiary university hospital in Brazil. METHODS: Thirteen cases of AOSD were identified in the Arthritis Outpatients Unit - Unicamp. Their clinical records were reviewed retrospectively in order to determine clinical manifestations regarding disease onset, follow-up and prescription. RESULTS: The prevalence of AOSD was 4.3%. The mean age of disease onset was 30.8 years old with a slight prevalence of men (54.2%). Constitutional symptoms, fever and cutaneous rash were observed in all cases. Hepatic involvement was observed in 11 patients, splenomegaly in 4, hematuria in 4, cardiac involvement in 2 and pleuritis in 2. Poliarticular involvement was observed more frequently, and 50% of them presented carpal ankylosis after 3 years of follow-up. Ferritine was elevated in 9 of 13 patients during active disease. All patients used oral non-steroidal anti-inflammatory drugs, steroids and 7 patients needed methotrexate. CONCLUSIONS: Although AOSD is a rare multisystemic rheumatic disorder, it should always be considered in febrile cases with poliarthritis. Neoplasia and infection should always be excluded. Normally it is characterized by a chronic course and carpal ankylosis as the main disabling feature.

adult-onset Still disease; diagnosis; prognosis


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