Current clinical aspects of familial Mediterranean fever

Abstract

The article is devoted to the analysis of the current state of the problem of the most common autoinflammatory disease – familial Mediterranean fever (FMF). The authors provide data on the prevalence of FMF, the genetic mechanisms of its development, the influence of MEFV gene mutations on the manifestation of FMF and its clinical phenotypes. The polymorphism of the mutated MEFV gene in various ethnic populations is discussed. Particular attention is paid to the combination of FMF with other rheumatic processes, primarily with spondyloarthritis. It has been shown that immunoinflammatory comorbidity is characteristic of the early onset of FMF, a more severe course of this disease, and is determined by the specific genotype of the patient. In this regard, the importance of genetic analysis is emphasized not only for the timely verification of FMF, but also for determining the prognosis in terms of the risk of developing spondyloarthritis and amyloidosis. An analysis of works on the pharmacotherapy of FMF showed that although colchicine remains the first-line drug, interleukin-1 inhibitors are actively used in the treatment of patients with FMF. The results of multicenter studies demonstrate the high efficacy and safety of the intelekin-1 inhibitor canakinumab in the treatment of FMF and other autoinflammatory diseases in children and adults. In this regard, with the development of colchicine resistance or poor tolerability of colchicine, interleukin-1 inhibitors are considered as the optimal therapeutic option.