mrjСовременная ревматологияModern Rheumatology Journal1996-70122310-158XIMA-PRESS, LLC10.14412/1996-7012-2023-1-78-82mrj-1392Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL OBSERVATIONSСочетание спорадического миозита с включениями и болезни Шегрена: обзор литературы и описание случаяCombination of sporadic inclusion body myositis and primary Sjögren’s syndrome: clinical case and review of literaturehttps://orcid.org/0000-0003-2314-1466ХванЮ. И.KhvanYu. I.

 Юлия Иннокентиевна Хван

 Россия, 115522, Москва, Каширское шоссе, 34А 

 Yulia Innokentievna Khvan 

 34A, Kashirskoe Shosse, Moscow 115522, Russia 

julija.hwan@gmail.comhttps://orcid.org/0000-0002-4804-3689Хелковская-СергееваА. Н.Khelkovskaya-SergeevaA. N.

 Россия, 115522, Москва, Каширское шоссе, 34А 

 34A, Kashirskoe Shosse, Moscow 115522, Russia 

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»РоссияV.A. Nasonova Research Institute of RheumatologyRussian Federation20231602202317178–82Copyright © Хван Ю.И., Хелковская-Сергеева А.Н., 20232023Хван Ю.И., Хелковская-Сергеева А.Н.Khvan Y.I., Khelkovskaya-Sergeeva A.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://mrj.ima-press.net/mrj/article/view/1392

В статье приведены обзор литературы и клиническое наблюдение пациентки с длительно текущей болезнью Шегрена (БШ) в сочетании со спорадическим миозитом с включениями (СМВ). Диагноз БШ утвержден в соответствии с российскими критериями БШ 2001 г., а также международными критериями ACR 2012 г. и ACR/EULAR 2016 г. Диагноз СМВ был установлен на основании характерной клинической картины: развитие заболевания у женщины после 50 лет с медленно прогрессирующей асимметричной мышечной слабостью и типичным распределением, умеренное повышение уровня креатинфосфокиназы (<10 норм за весь период наблюдения), наличие генерализованного первично-мышечного процесса по данным игольчатой электромиографии, типичной картины поражения мышц по данным магнитно-резонансной томографии и неэффективности высоких доз глюкокортикоидов. Отсутствие гистологического подтверждения не противоречит диагнозу, так как при морфологическом исследовании мышц у больных с типичным течением заболевания в 20% случаев не удается обнаружить характерные признаки СМВ.В настоящее время эффективная патогенетическая терапия СМВ отсутствует. Понимание механизмов развития СМВ позволит разработать действенные методы его лечения.

The article presents a review of the literature and a clinical observation of a patient with long-term anamnesis of primary Sjögren's syndrome (SS) in combination with sporadic inclusion body myositis (sIBM). The diagnosis of SS was confirmed in accordance with the Russian diagnostic criteria for SS 2001, as well as with the ACR 2012 and ACR/EULAR 2016 criteria. The diagnosis of sIBM was established on the basis of a characteristic clinical picture: the development of the disease in a woman after 50 years of age with slowly progressive asymmetric muscle weakness and a typical distribution, a moderate increase in the level of creatine phosphokinase (<10 norms for the entire observation period), the presence of a generalized primary muscle process according to needle electromyography, a typical picture of muscle involvement according to magnetic resonance imaging, and the ineffectiveness of high doses of glucocorticoids. The absence of histological confirmation does not contradict the diagnosis, since morphological examination of muscles in patients with a typical course of the disease fails to detect characteristic signs of sIBM in 20% of cases.

Currently, there is no effective pathogenetic therapy for sIBM. Understanding the mechanisms of sIBM development will allow to develop effective methods of its treatment.

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