Adult-onset Still's disease: clinical, serological and therapeutic considerations.

OBJECTIVES

This paper aims to describe the clinical manifestations, laboratory abnormalities and treatment of adult-onset Still's disease (AOSD) in Greek patients.

METHODS

This is a retrospective observational study. Forty-four patients, diagnosed with AOSD, followed since 1985 up to June 2011, were included. The disease course and treatment were recorded and compared to previously published studies.

RESULTS

Twenty-one males and 23 females were identified. Mean age at diagnosis was 38.3 years. The most common clinical manifestations were fever (100%), arthralgias (97.7%), arthritis (93.2%), salmon-coloured rash (84%), myalgias (50%) and sore throat (38.6%). Characteristic laboratory abnormalities were leucocytosis with neutrophilia (81.8%), elevated C-reactive protein (100%) and erythrocyte sedimentation rate (100%). Elevated liver enzymes and hyperferritinaemia were found in 50% and in 59% of the patients respectively. Very high ferritin serum levels (>5000 μg/l) were found in 22.7%. Rheumatoid factor and antinuclear antibodies were negative in all patients. Thirty patients (68.2%) received non-steroidal anti-inflammatory drugs or aspirin with or without corticosteroids. Response to corticosteroids was common (58.9%). When this treatment was ineffective, a disease-modifying anti-rheumatic drug (DMARD), usually methotrexate, was added with a response rate of 63.6%. Anakinra was used in cases resistant to conventional immunosuppressive treatment. Ten out of 44 patients (22.7%) were treated with anakinra and response was achieved in all of them.

CONCLUSIONS

Our results regarding clinical manifestations and laboratory abnormalities were similar to those of previous reports. High ferritin serum levels were reported in all studies of AOSD and are considered as diagnostically valuable. When treatment with corticosteroids and DMARDS had failed, biologic agents such as anakinra were successfully applied.