avkАрхивъ внутренней медициныThe Russian Archives of Internal Medicine2226-67042411-6564“SINAPS” LLC10.20514/2226-6704-2018-8-3-176-185avk-785Research ArticleОБЗОРНЫЕ СТАТЬИREVIEW ARTICLESИММУНОПАТОГЕНЕЗ МИАСТЕНИИ ГРАВИС (ОБЗОР ЛИТЕРАТУРЫ)IMMUNOPATHOGENESIS OF MYASTHENIA GRAVIS (REVIEW)ГасымлыЭ. Д.GasymlyE. D.

Department of Nervous Diseases with a medical rehabilitation course

elya_qasimli@mail.ruФедеральное государственное бюджетное образовательное учреждение высшего образования «Красноярский государственный медицинский университет имени профессора В.Ф. Войно-Ясенецкого» Министерства здравоохранения Российской Федерации, кафедра нервных болезней с курсом медицинской реабилитации ПО, КрасноярскРоссияFederal State Educational Institution of Higher Education «Krasnoyarsk State Medical University named after Professor V.F. Voyno-Yasenetsky» of the Russian Federation Ministry of Health, KrasnoyarskRussian Federation20180406201883176185Copyright © Гасымлы Э.Д., 20182018Гасымлы Э.Д.Gasymly E.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.medarhive.ru/jour/article/view/785

Миастения гравис — тяжелое прогрессирующее аутоиммунное заболевание, характеризующееся выработкой антител к структурам нервно-мышечного соединения. Высокая клиническая гетерогенность аутоиммунной миастении и инициализирующее течение повышают актуальность изучения ее патогенеза, поиска специфичных методов маркерной диагностики, разработки алгоритмов для прогнозирования особенностей развития заболевания. На сегодняшний день существуют разные подходы в изучении этиологии и патогенеза заболевания, в числе которых серологические, биохимические и генетические теории развития болезни. На протяжении десятков лет ведутся исследования, направленные на поиск новых патогенетических звеньев миастении гравис. На сегодняшний день уже описан ряд антител, которые возможно применять при диагностике миастении гравис; серологическая диагностика миастении стала применяться в клинической практике в качестве «золотого стандарта». Уже разработаны прогностические критерии, описывающие характер течения миастении гравис и типы антител, выделенных в сыворотке крови пациента, описаны механизмы срыва аутотолерантности, запускающие выработку антител к собственным структурам, их генетические основы. С развитием биотехнологических методов, исследователи смогли идентифицировать подтип лимфоцитов, участвующих в развитии миастении. Стало доступно выделение отдельных субпопуляций лимфоцитов. Исследователи продолжают поиски новых мишеней, которые позволят усовершенствовать диагностику миастении гравис, разработать новые направления в терапии заболевания. Однако несмотря на активное изучения различных механизмов развития миастении, еще остается множество нерешенных задач. В статье кратко описаны основные изученные механизмы развития миастении гравис, что облегчит практикующим врачам понимание сложных механизмов ее патогенеза.

Myasthenia gravis is a progressive autoimmune disease, which characterized by the production of antibodies to the structures of the neuromuscular junction. High clinical heterogeneity of autoimmune myasthenia, initiating the flow, increases the urgency of studying its pathogenesis, searching for specific methods of marker diagnostics, developing algorithms for predicting the features of the development of the disease. At the present time, there are different approaches to the study of the etiology and pathogenesis of the disease, which include both serological, biochemical, genetic, etc. theory of the development of the disease. For decades, research has been carried out to find new pathogenetic links in myasthenia gravis. Today was described a number of antibodies such as for muscarin (MuSK), ryanodine receptors, to titin, lipoprotein bound receptor 4, cortactin, etc.). The serological diagnosis of myasthenia gravis has been used as a «gold standard» in clinical practice. The prognostic criteria describing the course of myasthenia gravis and the type of antibodies isolated in the blood serum of the patient. Also, already have been developed mechanisms of failure of autotolerance, triggering the production of antibodies to their own structures, and their genetic bases are also described. Thanks to the development of biotechnological methods, the researchers were able to identify the subtype of lymphocytes involved in the development of myasthenia gravis. Isolation of individual subpopulations of lymphocytes also became available. Researchers continue to search for new targets, allowing to improve diagnostics, to develop new directions in the therapy of the disease. However, despite the active study of various mechanisms for the development of myasthenia gravis, many unresolved problems still remain. The article describes briefly the main mechanisms studied in the development of myasthenia gravis, which in turn facilitates understanding of complex mechanisms of pathogenesis.

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