rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.47360/1995-4484-2023-385-388rsp-3367Research ArticleКЛИНИЧЕСКИЙ СЛУЧАЙCLINICAL OBSERVATIONСиндром Сусака: эффективность монотерапии ритуксимабомSusac syndrome: The effectiveness of Rituximab monotherapyhttps://orcid.org/0000-0003-2641-9785БекетоваТ. В.BeketovaT. V.

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

tvbek@rambler.ruФГБУ «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 Federation202329062023613385388Copyright © Бекетова Т.В., 20232023Бекетова Т.В.Beketova T.V.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/3367

Синдром Сусака (СС), или ретикуло-кохлео-церебральньная васкулопатия, является крайне редким тяжелым инвалидизирующим заболеванием, в основе которого предполагают окклюзирующую микроангиопатию. Для СС характерна клиническая триада, включающая энцефалопатию, нейросенсорную тугоухость и окклюзию артерий сетчатки. Лечение СС недостаточно разработано; используют высокие дозы глюкокортикоидов, внутривенный человеческий иммуноглобулин циклофосфан и анти-В-клеточную терапию ритуксимабом (РТМ). Представлено описание случая СС с типичными клиническими проявлениями и волнообразным, медленно прогрессирующим течением заболевания, при котором отмечена эффективность монотерапии РТМ. Обсуждается целесообразность использования монотерапии РТМ на ранних стадиях СС, в случаях медленно прогрессирующего течения заболевания, при наличии противопоказаний для применения глюкокортикоидов и цитостатиков.

Susac syndrome (SS) or retino-cochleo-cerebral vasculopathy is an extremely rare, severe, and potentially disabling condition. Underlying occlusive microangiopathy in SS is clinically characterized by the triad of encephalopathy, sensorineural hearing loss and branch retinal arterial occlusion. SS therapy envisages simultaneous use of high doses of glucocorticoids, intravenous immunoglobulins, cyclophosphamide and rituximab (RTХ). This article presents a case of remitting-relapsing slow-progressive SS with typical clinical manifestations demonstrating successful treatment SS with RTХ monotherapy; it also discuss the focus of RTХ monotherapy should be targeted at SS cases with contraindications for glucocorticoids and cytostatics use, slow-progressive SS or at early stages.

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