THU0583 EXPERIENCE OF ANAKINRA AND CANAKINUMAB IN PATIENTS WITH COLCHICINE-RESISTANT FAMILIAL MEDITERRANEAN FEVER AND COMPLICATED WITH AMYLOIDOSIS

2019 
Background: Familial Mediterranean Fever (FMF) is a hereditary auto-inflammatory disease characterized by recurrent fever and serosal inflammation (1). The goal of FMF treatment is to prevent the attacks and to minimize subclinical inflammation between attacks, and in attacks-free period. Colchicine is a major drug that sine qua non in the treatment of FMF. However, anti-interleukin-1 agents are recommended in colchicine resistant and/or intolerant FMF patients (2). Objectives: The aim of this study is to evaluate the efficacy of anti-interleukin-1 (anti-IL-1) agents in 54 FMF patients with resistant/intolareted to colchicine or complicated with amyloidosis. Methods: Between January 2014 and December 2018, fifty four patients who were diagnosed as FMF according to the criteria of Tel-Hashomer that following-up at Sivas Cumhuriyet University Medical Faculty, Rheumatology-Internal Medicine Department were included in to the study. Results: 25 (46.3%) male and 29 (53.7%) female were included in the study. The median age of the patients was 29 (min:17-max: 60) years and the median age at diagnosis was 18 (min: 3-max: 46) years. Anakinra was used in 34 (63%) FMF patients (100 mg/day), and canakinumab was used in 20 (37%) patients (150mg/8weeks). 37 cases were resistant to colchicine, 8 were intolerant to colchicine, 9 (20%) cases were comlicated with amyloidosis. 8 patients had renal transplantation. MEFV gene mutations are shown in Table 1. Median duration of anti-IL-1 agent use was 12 months (min:2-max40). 5 patients were resistant to anakinra, 4 patients had side effects which anakinra related. After a median follow up 12 months overall clinical response was%95 (frequency of attacks Conclusion: Anti-interleukin-1 agents can be used effectively and safely in the treatment of FMF patients. These agents are especially effective in the treatment of proteinuria due to amyloidosis. Large and long-lasting follow-up studies are needed to evaluate long-term effects of these drugs. References: [1] Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. 2019Jan24. pii: S0755-4982(18)30486-X. doi: 10.1016/j.lpm.2018.08.014. [Epub ahead of print]. [2] Seza Ozen ve ark. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016Apr;75(4):644-51. Disclosure of Interests: None declared
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