vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v17i5.1953vsp-1953Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWСемейная средиземноморская лихорадка: современные подходы к диагностике и лечениюFamilial Mediterranean Fever: Current Approaches to Diagnosis and TreatmentКостикМ. М.KostikMikhail M.

Костик Михаил Михайлович - доктор медицинских наук, доцент, и.о. заведующего кафедрой госпитальной педиатрии СПбГПМУ.

194100, Санкт-Петербург, ул. Литовская, д. 2; тел.: +7 (812) 416-52-98

kost-mikhail@yandex.ruhttps://orcid.org/0000-0002-2336-6246ЖоговаО. В.ZhogovaOlga V.https://orcid.org/0000-0001-5296-2752ЛагуноваН. В.LagunovaNatalia V.https://orcid.org/0000-0001-8814-1948ИвановскийС. В.IvanovskiySergey V.

Симферополь.

https://orcid.org/0000-0002-6980-8046КолобоваО. Л.KolobovaOksana L.

194100, Санкт-Петербург, ул. Литовская, д. 2; тел.: +7 (812) 416-52-98

https://orcid.org/0000-0002-6437-2546МельниковаЛ. Н.MelnikovaLarisa N.

194100, Санкт-Петербург, ул. Литовская, д. 2; тел.: +7 (812) 416-52-98

Санкт-Петербургский государственный педиатрический медицинский университетРоссияSt. Petersburg State Pediatric Medical UniversityRussian FederationКрымский федеральный университет им. В.И. Вернадского; Республиканская детская клиническая больницаРоссияV.I. Vernadskiy Crimean Federal University; Republican Pediatric Clinical HospitalRussian FederationКрымский федеральный университет им. В.И. ВернадскогоРоссияV.I. Vernadskiy Crimean Federal UniversityRussian Federation201801122018175371380Copyright © Костик М.М., Жогова О.В., Лагунова Н.В., Ивановский С.В., Колобова О.Л., Мельникова Л.Н., 20182018Костик М.М., Жогова О.В., Лагунова Н.В., Ивановский С.В., Колобова О.Л., Мельникова Л.Н.Kostik M.M., Zhogova O.V., Lagunova N.V., Ivanovskiy S.V., Kolobova O.L., Melnikova L.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://vsp.spr-journal.ru/jour/article/view/1953

Семейная средиземноморская  лихорадка  — типичное моногенное  заболевание  с аутосомно-рецессивным типом наследования;  обусловлено  мутациями в гене MEFV, кодирующем белок пирин. Является сравнительно редкой патологией  в практике  педиатров  и ревматологов  Российской  Федерации.  В статье  приводятся  современные данные о распространенности  заболевания,  представлена  полная на сегодняшний день клиническая картина аутовоспалительного синдрома, рассматриваются диагностические критерии и методы лечения пациентов с семейной средиземноморской лихорадкой.

Familial Mediterranean fever is a typical monogenic disease with an autosomal recessive inheritance pattern; caused by mutations in the MEFV gene, which encodes the pyrin protein. It is a relatively rare pathology in the practice of paediatricians and rheumatologists of the Russian Federation. The article provides up-to-date data on the disease prevalence, presents a complete clinical picture of the auto-inflammatory  syndrome, discusses diagnostic criteria and methods for treating patients with familial Mediterranean fever.

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