Background Approximately 5 to10% of FMF patients do not respond to colchicine treatment and/or intolerant to colchicine because of side effects. Several case reports and case series have pointed out the efficacy of IL-1 blockade in colchicine resistant FMFsubgroup.
Objectives To review the patients followed in our center with FMF who received anakinra, an anti IL-1 receptor antagonist, because of insufficient colchicine response.
Methods FMF patients who were treated with anakinra were retrospectively reviewed with regard to indication, effect on disease activity and acute phase response, adverse events. Patient global assessment was recorded before and after anakinra treatment.
Results There were 30 FMF patients with FMF who were treated with anakinra for various indications (amyloidosis in 7, colchicine resistant recurrent febrile attacks in 19, colchicine related side effects in 4). The mean age of this group was 36.34±11.64 years. The mean duration of the disease was 23.93±12 years. There were various co-existing pathologies among this study group like multiple sclerosis (1), ankylosing spondylitis (1), SLE (1), Behçet's disease (1), low grade lymphoma (1) and PAN (2). Four patients were pregnant. The mean colchicine dose was 2,08±0,5 mg/d. The mean duration of anakinra treatment was 13,5±12,93 months. Twenty three patients reported no attacks after anakinra treatment whereas 5 patients reported at least 50% decrease in the attack frequency. Mean patient global assessment decreased from 8,60±2,3 to 1,92±2,7 under anakinra treatment (p=0.001).
Among the 7 patients with amyloidosis, anakinra was stopped in 2 patients because of increased proteinuria. However, a significant decrease in proteinuria was detected in 3 patients. One patient developed severe injection site reaction and therefore the drug was stopped. Overall, 4 of our patients with amyloidosis are still on Anakinra treatment.
Three patients had a severe allergic reaction (severe disseminated rash in 1 patient and severe injection site reaction in 2 patients) and therefore the drug was stopped. Two patients had infections (one had genital warts and urinary tract infection, the other had sinusitis and folliculitis) and the treatment was terminated. One of our patients reported that her psoriatic lesions got worse on anakinra. Twenty two patients reported no adverse events during the treatment.
Conclusions Anakinra was effective in controlling the symptoms in colchicine-resistant FMF cases. It was also effective in FMF related amyloidosis. The major cause of treatment termination was injection site reactions. Anakinra seems to be an effective alternative in patients who have insufficient response to colchicine.
Disclosure of Interest None declared