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The IL-1 inhibitor Canakinumab for Familial Mediterranean Fever: the Greek experience in 12 patients
Pediatric Rheumatology volume 13, Article number: P72 (2015)
IL-1 is a major mediator of the inflammatory cascade in Familial Mediterranean Fever (FMF) and an established therapeutic target .
To retrospectively assess the efficacy and safety of the IL-1 inhibitor Canakinumab in adult and adolescent FMF patients, including cases resistant to the IL-1 receptor antagonist anakinra.
Twelve patients (7 men) with genetically confirmed FMF, fulfilling the Tel Hashomer criteria, aged 32.5 years (median, range 13-70), with median disease duration of 168 months and active disease refractory to colchicine (n=8) and/or anakinra (n=4), received Canakinumab 150mg subcutaneously every 4 (n=7) or 6 (n=3) or 8 weeks (n=2) for a median of 12 months (range 4-46). Canakinumab was given as monotherapy in 9; 3 patients received concomitant treatment with colchicine and/or corticosteroids. Clinical and laboratory parameters during follow-up were recorded.
Seven out of 12 patients (58%) achieved complete clinical remission within a median time of one month. Normalization of all laboratory parameters associated with inflammation occurred in 70% of patients within a median time of 2 months and in all, but one, of these patients complete clinical remission was also reached. Response was maintained until the last visit in all patients with clinical and/or serological complete remission. The remaining patients achieved partial responses, with persisting, albeit milder, arthralgias and abdominal pain, and lower, but abnormal CRP levels. Overall, the concomitant corticosteroid dose was significantly reduced during follow up. The recently proposed FMF50 score for assessing outcome in FMF (2) was achieved by 67% and 88% of patients at one month and 12 months, respectively. Canakinumab was well tolerated; one patient experienced an urinary tract infection, which resolved with antibiotics.
The rapid and sustained response to Canakinumab in the majority of our patients, together with the favorable safety profile, encourages its further use in FMF.
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