Background/Purpose: Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Familial Mediterranean Fever (FMF) and an established therapeutic target (1). To retrospectively assess the efficacy and safety of the IL-1 inhibitor Canakinumab in adult and adolescent FMF patients.

Methods: Thirteen patients (7 men) with genetically confirmed FMF, fulfilling the Tel Hashomer criteria, aged 37 years (median, range 13-70), with median disease duration of 15 years and active disease despite treatment with colchicine (n=9), anakinra (n=1) or both (n=3), received Canakinumab 150mg subcutaneously every 4 (n=7) or 6 (n=2) or 8 weeks (n=4) for a median of 18 months (range 7-53). Canakinumab was given as monotherapy in 9; 4 patients received concomitant treatment with colchicine and/or corticosteroids. The clinical and laboratory parameters during follow up were recorded.

Results: Ten out of 13 patients (77%) achieved complete clinical remission within a median time of 3.5 months, while normalization of all laboratory parameters associated with inflammation occurred in 85% of patients within a median time of 3 months. The remaining patients achieved partial responses, with persisting, albeit milder, arthralgias, exertional leg pain, abdominal pain, anemia, and lower, but abnormal, C-reactive protein levels. Response was maintained until the last visit in all but one patient who relapsed with fever and arthralgias and re-remitted after reducing the Canakimumab administration interval. Overall, in 3 patients, including the patient who relapsed, the interval between Canakinumab injections was reduced in order to achieve complete remission, whereas in another two patients drug administration intervals could be safely increased without disease exacerbation until the last visit. The concomitant corticosteroid dose was significantly reduced during follow up. The recently proposed FMF50 score for assessing outcome in FMF (2) was achieved by 62% and 85% of patients at one month and 12 months, respectively. Canakinumab was well tolerated; one patient experienced an urinary tract infection, which resolved with antibiotics, and another one a viral gastroenteritis, which required short-term hospitalization.

Conclusion: 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.

1. Ter Haar N et al. Ann Rheum Dis. 2013;72(5):678-85.

2. Ozen S et al. Ann Rheum Dis. 2014;73(5):897-901.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-interleukin-1-inhibitor-canakinumab-for-familial-mediterranean-fever-long-term-beneficial-effect-in-a-cohort-of-13-patients/