The efficacy of canakinumab in the treatment of a patient with familial Mediterranean fever and longstanding destructive arthritis.

摘要

Self-limited, non-destructive arthritis is a common clinical presentation of familial Mediterranean fever (FMF), while protracted, refractory to standard treatment arthritis is a rare manifestation of the disease, potentially resulting in severe damage and disability. We report on a 25-year-old woman with FMF, homozygous for the MEFV gene M694V mutation, and with longstanding articular involvement affecting her hips and her left knee, effectively treated with canakinumab. She had been under treatment with colchicine since the age of 5. In 2005, she experienced long-lasting arthritis of her right hip and treatment with anakinra (100 mg/day) was added. Anakinra was discontinued shortly afterwards due to severe injection site reactions, precluding the evaluation of its efficacy. She was switched to treatment with etanercept (25 mg twice weekly) and low dose prednisone (5-7.5 mg/day). In 2008, she developed destructive arthritis of the right hip, which lead to total hip replacement, and chronic arthritis of her left knee. Methotrexate (10 mg/ week) was added to the existent treatment. Long-term remission was not achieved, as indicated by the clinical findings and the elevated inflammatory parameters (erythrocyte sedimentation rate (ESR) and C reactive protein (CRP)) during the follow-up period. She described five crises of FMF, during the following year.