nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-1004Research ArticleШКОЛА НЕФРОЛОГАEDUCATIONAL MATERIALSКатастрофический антифосфолипидный синдром у молодой больной системной красной волчанкойCatastrophic antiphospholipid syndrome in young woman with the systemic lupus erythematosusКозловскаяН. Л.KozlovskayaN. L.nkozlovskaya@yandex.ruТомилинаН. А.TomilinaN. A.noemail@neicon.ruСтоляревичЕ. С.StolyarevichE. S.noemail@neicon.ruБирюковаЛ. С.BirukovaL. S.noemail@neicon.ruСкрябинаИ. А.SkryabinaI. A.noemail@neicon.ruКальяноваЕ. В.KalyanovaE. V.noemail@neicon.ruВарясинВ. В.VaryasinV. V.noemail@neicon.ruЖуковаМ. Е.ZhukovaM. E.noemail@neicon.ruКафедра нефрологии и гемодиализа ММА им. И.М. СеченоваРоссияMoscow Sechenov Medical Academy, chair of nephrology and hemodialysisRussian FederationФГУ «ФНЦ трансплантологии и искусственных органов имени акад. В.И. Шумакова» Минздравсоцразвития РФ, г. МоскваРоссияShumakov Federal Research Center of Transplantology and Artificial Organs, MoscowRussian FederationГКБ № 52, г. МоскваРоссияMoscow City Hospital № 52Russian FederationКафедра нефрологии ФПДО МГМСУРоссияMoscow State University of Medicine and Dentistry, Russian Federal Health AgencyRussian Federation201017062025123208219Copyright © Козловская Н.Л., Томилина Н.А., Столяревич Е.С., Бирюкова Л.С., Скрябина И.А., Кальянова Е.В., Варясин В.В., Жукова М.Е., 20252025Козловская Н.Л., Томилина Н.А., Столяревич Е.С., Бирюкова Л.С., Скрябина И.А., Кальянова Е.В., Варясин В.В., Жукова М.Е.Kozlovskaya N.L., Tomilina N.A., Stolyarevich E.S., Birukova L.S., Skryabina I.A., Kalyanova E.V., Varyasin V.V., Zhukova M.E.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/1004

В статье приводятся сведения о КАФС, в том числе основные концепции патогенеза, клинические проявления, морфологические особенности, подходы к диагностике и лечению, подчеркиваются его отличия от классических вариантов АФС – первичного и вторичного при СКВ. Обзор иллюстрирован клиническим наблюдением больной КАФС, развившимся как основное проявление СКВ. На примере представленного случая разбираются некоторые аспекты дифференциальной диагностики КАФС с другими микроангиопатическими синдромами – ГУС/ТТП и ДВС.

A review of literature on catastrophic antiphospholipid syndrome (CAS) including the basic concepts of pathogenesis, clinical displays, morphological features, diagnostics and treatment is presented. The difference between classical variants of CAS – primary and secondary, at systemic lupus erythematosus, is emphasized. The review is illustrated by a clinical case of a patient with CAS which has developed as the main manifestation of SLE. Some aspects of differential diagnostics of CAS with others microangiopathic syndromes – GUS/TTP and DIC-syndrom – are presented.

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