medgenМедицинская генетикаMedical Genetics2073-7998Publishing House «Genius Media» LLC10.1234/XXXX-XXXX-2016-3-3-11medgen-99Research ArticleНАУЧНЫЕ ОБЗОРЫREVIEWСемейная средиземноморская лихорадка: современные представленияFamilial mediterranean fever: current viewАтоянС. А.AtoyanS. A.tamsar@sci.amЦентр медицинской гeнетики и первичной охраны здоровья; Ереванский Государственный Медицинский Универстет им. Мхитара ГерациРоссияCenter of Medical Genetics and Primary Health Care; Yerevan State Medical University after Mkhitar HeratsiRussian Federation201607102016153311Copyright © Атоян С.А., 20162016Атоян С.А.Atoyan S.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.medgen-journal.ru/jour/article/view/99

Семейная средиземноморская лихорадка, или периодическая болезнь (ПБ), - наследственное аутовоспалительное заболевание, наследуемое по аутосомно-рецессивному типу. Причина развития ПБ - мутации гена MEFV , который локализуется на коротком плече хромосомы 16. При ПБ наблюдаются периодически повторяющиеся приступы лихорадки, сопровождающиеся серозитами разной локализации, артритами, эризипелоидоподобным высыпанием; наиболее тяжелым осложнением является амилоидоз почек. Постановка диагноза базируется на клинических критериях и результатах генетического анализа мутаций гена MEFV . Основным медикаментом, позволяющим уменьшить частоту приступов и риск развития осложнений, в том числе и амилоидоза почек, является колхицин. В основе патогенеза ПБ лежит гиперактивация естественного иммунитета в виде гиперсекреции интерлейкинов, и препараты, блокирующие функции интерлейкинов, эффективны прежде всего у пациентов с побочными эффектами или резистентных к колхицинотерапии. В обзоре обсуждаются современные этиологические факторы заболевания, обусловленного генными мутациями, приводятся сведения о молекулярно-генетических методах диагностики, прогнозе развития заболевания, а также коррекции лечения.

Familial Mediterranean fever (FMF ) is the hereditary autoinflammatory disease with autosom-recessve way of inheritance. The gene MEFV is responsible for the development of the disease, which is located on the short arm of 16th chromosome. The clinical picture of FMF is characterized by periodically repeated episodes of fever, poliserositis of different localizations, and rarely by erysipelas-like skin rash. The most severe complication of FMF is renal amyloidosis. The diagnosis of FMF is made based on the typicl clinical criteria and genetic testing of MEFV gene mutations. Colchicine is the main medication which significantly decreases the number of FMF attacks and the possibility of renal amyloidosis development. However other medications blocking the interleukins are effective in cases of adverse effects or resistency to colchicines, since the hyperactivation of innate immune system and hypersecretion of interleukins are underlied of FMF pathogenesis. In this review the issues conserning the modern view of etiology of FMF associated with MEFV gene mutations, the new approaches of molecular-genetic diagnosis, and further prognosis are discussed.

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The authors declare that there are no conflicts of interest present.