References

rsp

Научно-практическая ревматология

Rheumatology Science and Practice

1995-44841995-4492

IMA-PRESS, LLC

10.47360/1995-4484-2023-458-465

rsp-3400

Research Article

ПРОГРЕСС В РЕВМАТОЛОГИИ В XXI ВЕКЕ

PROGRESS IN RHEUMATOLOGY IN THE XXI CENTURY

Моногенные аутовоспалительные синдромы с чертами системных васкулитов: новая область ревматологии

Monogenic autoinflammatory syndromes with features of systemic vasculitis: a new field of rheumatology

https://orcid.org/0000-0003-2641-9785

Бекетова

Т. В.

Beketova

T. V.

д.м.н., зав.отделением «ревматологии с нефрологическими койками и кабинетом терапии ГИБП» ФГБУ «Центральная клиническая больница с поликлиникой» Управления делами Президента Российской Федерации, ведущий научный сотрудник лаборатории «микроциркуляции и воспаления» ФГБНУ НИИР им. В.А. Насоновой, профессор кафедры «инфокогнитивные технологии» факультета информационных технологий ФГАОУ ВО «Московский политехнический университет»

121356, Москва, ул. Маршала Тимошенко, 15 115522, Москва, Каширское шоссе, 34а 107023, Москва, ул. Большая Семёновская, 38

121359, Moscow, Marshala Timoshenko str., 19, building 1A 115522, Moscow, Kashirskoye Highway, 34A 107023, Moscow, Bolshaya Semyonovskaya str., 38

https://orcid.org/0000-0001-5562-0969

Бекетова

М. Ф.

Beketova

M. F.

клинический ординатор кафедры «внутренних болезней» факультета фундаментальной медицины

119234, Москва, Ленинские горы, 1

119234, Moscow, Leninskie Gory, 1

beketova_maria@rambler.ru

https://orcid.org/0000-0002-1598-8360

Насонов

Е. Л.

Nasonov

E. L.

д.м.н, профессор, академик РАН, научный руководитель

115522, Москва, Каширское шоссе, 34а

119991, Москва, ул. Трубецкая, 8, стр. 2

115522, Moscow, Kashirskoye Highway, 34A

119991, Moscow, Trubetskaya str., 8, building 2

ФГБУ «Центральная клиническая больница с поликлиникой» Управления делами Президента Российской Федерации; ФГБНУ «Научно- исследовательский институт ревматологии им. В.А. Насоновой»; ФГАОУ ВО «Московский политехнический университет»РоссияCentral State Medical Academy of the Administrative Directorate of the President of the Russian Federation; V.A. Nasonova Research Institute of Rheumatology; Moscow Polytechnic UniversityRussian Federation

ФГБОУ ВО «Московский государственный университет имени М.В. Ломоносова»РоссияLomonosov Moscow State UniversityRussian Federation

ФГБНУ «Научно-исследовательский институт ревматологии им. В.А. Насоновой»; ФГАОУ ВО «Первый Московский государственный медицинский университет имени И.М. Сеченова» Минздрава России (Сеченовский Университет)РоссияV.A. Nasonova Research Institute of Rheumatology; I.M. Sechenov First Moscow State Medical University of the Ministry of Health Care of Russian Federation (Sechenov University)Russian Federation

2023

31082023

614458465

2023

Бекетова Т.В., Бекетова М.Ф., Насонов Е.Л.

Beketova T.V., Beketova M.F., Nasonov E.L.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://rsp.mediar-press.net/rsp/article/view/3400

Обзор посвящен новой актуальной проблеме ревматологии – моногенным аутовоспалительным синдромам с чертами системных васкулитов, которые могут манифестировать во взрослом возрасте. Рассматриваются особенности клинического течения редко диагностируемого синдрома VEXAS, а также встречающихся иногда у взрослых синдромов SAVI и СОРА. Обсуждаются перспективные направления лечения будущего.

The article is dedicated to a new actual problem in rheumatology: vasculitis and vasculitis-like manifestations in monogenic autoinflammatory syndromes in adult. The features of the clinical course of the rarely diagnosed VEXAS syndrome, as well as the SAVI and COPA syndromes, which sometimes occur in adults, are considered. Promising directions of future treatment are discussed.

синдром VEXASсиндром SAVIсиндромСОРАсистемный васкулитмоногенный аутовоспалительный синдромваскулопатия

syndrome VEXASsyndrome SAVIsyndrome COPAsystemic vasculitismonogenic autoinflammatory syndromevasculopathy

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