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Современная ревматология

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Смешанное заболевание соединительной ткани

https://doi.org/10.14412/1996-7012-2019-1-11-18

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Аннотация

Смешанное заболевание соединительной ткани (СМЗСТ; синдром Шарпа) — редкое системное заболевание соединительной ткани, характеризующееся сочетанием отдельных признаков системной красной волчанки, системной склеродермии, ревматоидного артрита, полимиозита с наличием антител к растворимому ядерному рибонуклеопротеину (анти-и1-РНП) в высоких титрах. Кнаи-более частым клиническим проявлениям СМЗСТ относят феномен Рейно, отек кистей, мышечную слабость, артралгии/артриты, гипотонию пищевода. Течение заболевания преимущественно доброкачественное, однако имеются случаи тяжелого течения с поражением легких, почек, сердечно-сосудистой системы и ЦНС. Плохой прогноз и наибольшая смертность связаны с легочной артериальной гипертензией. Диагностика СМЗСТ затруднена в связи с отсутствием унифицированных диагностических критериев и специфических проявлений в дебюте заболевания. Кроме того, не существует общепринятых рекомендаций по лечению СМЗСТ.

В статье рассмотрены современные представления о СМЗСТ: имеющиеся критерии диагностики, клинические и иммунологические особенности, лечение.

Об авторах

Р. У. Шаяхметова
ФГБНУ Научно-исследовательский институт ревматологии им. В.А. Насоновой
Россия

115522, Москва, Каширское шоссе, 34А



Л. П. Ананьева
ФГБНУ Научно-исследовательский институт ревматологии им. В.А. Насоновой
Россия

Лидия Петровна Ананьева

115522, Москва, Каширское шоссе, 34А



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Для цитирования:


Шаяхметова Р.У., Ананьева Л.П. Смешанное заболевание соединительной ткани. Современная ревматология. 2019;13(1):11-18. https://doi.org/10.14412/1996-7012-2019-1-11-18

For citation:


Shayakhmetova R.U., Ananyeva L.P. Mixed connective tissue disease. Modern Rheumatology Journal. 2019;13(1):11-18. (In Russ.) https://doi.org/10.14412/1996-7012-2019-1-11-18

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ISSN 1996-7012 (Print)
ISSN 2310-158X (Online)