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.

Treatment with canakinumab (ACZ885, Ilaris; Novartis Pharma, Basel, Switzerland), a fully humanised monoclonal antibody targeting interleukin (IL)-1β, 4 5 (150 mg subcutaneously) was started in May 2010, after a washout period of 4 weeks for etanercept and methotrexate. Prednisone was discontinued 3 months afterwards. Treatment with colchicine (2 mg/day) was maintained. Our patient received four doses of canakinumab as follows: at the beginning of treatment and at weeks 8, 14 and 20 (figure 1). Pain and stiffness of the affected joints, global assessment score and acute phase reactants (CRP, ESR) were evaluated every 2 weeks over a 24-week period (figure 1). Radiological evaluation of the affected joints by MRI was performed before the initiation of the treatment and at week 24.