Open AccessTreatment of familial mediterranean fever with canakinumab in patients who are unresponsive to colchicine
Author(s) -
Afig Berdelı,
Özgür Şenol,
Gamze Talay
Publication year - 2019
Publication title -
european journal of rheumatology :/european journal of rheumatology
Language(s) - English
Resource type - Journals
eISSN - 2147-9720
pISSN - 2148-4279
DOI - 10.5152/eurjrheum.2019.18190
Subject(s) - canakinumab , familial mediterranean fever , medicine , colchicine , mefv , rheumatology , dermatology , anakinra , gastroenterology , pediatrics , disease , gene mutation , mutation , biochemistry , chemistry , gene
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.