Treatment of familial mediterranean fever with canakinumab in patients who are unresponsive to colchicine

European Journal of Rheumatology
Afig BerdeliGamze Talay

Abstract

Familial Mediterranean fever (FMF) is the most common inherited monogenic autoinflammatory disease worldwide. It is caused by loss-of-function mutations in the MEFV gene, mostly affecting Eastern Mediterranean population. It is discussed if it should be considered an autosomal-dominant disease with variable penetrance, because heterozygosis mutations are associated with clinical autoinflammatory manifestations. Colchicine constitutes that the mainstay of FMF treatment should be preventing acute attacks and amyloidosis, and decreasing the chronic inflammation. In colchicine-resistant or intolerant patients, recent insights into the pathogenesis of FMF have made the anti-IL1 treatments important. We aimed to search for the retrospective results of canakinumab treatment in patients with FMF who are unresponsive to colchicine. In this study, 22 (13 males and nine females) patients with FMF with colchicine resistance/intolerance, age ranging from 6 to 18 years, were included in Ege University Department of Pediatric Rheumatology. After clinical and genetic diagnosis, colchicine treatment with standard doses was started. After treatment with canakinumab, complete response to treatment was determined as no acute episodes and normal le...Continue Reading

Citations

Apr 8, 2020·Evidence-based Complementary and Alternative Medicine : ECAM·Sicong LiLei Bi
Jul 17, 2020·Frontiers in Immunology·Véronique HentgenSophie Georgin-Lavialle
Mar 10, 2021·NPJ Precision Oncology·Jeong-Hoon JangYoung-Joon Surh
Mar 21, 2021·Nefrología : publicación oficial de la Sociedad Española Nefrologia·Tolga YildirimYunus Erdem
May 12, 2021·Clinical Journal of Gastroenterology·Yoshihiro YokoyamaHiroshi Nakase

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