rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2018-249-252rsp-2532Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL OBSERVATIONSСиндром Сусака (ретино-кохлео-церебральная васкулопатия)SUSAC'S SYNDROME (RETINOCOCHLEOCEREBRAL VASCULOPATHY)БекетоваТ. В.BeketovaT. V.

Татьяна Валентиновна Бекетова.

115522, Москва, Каширское шоссе, 34А

Tatiana Beketova.

34A, Kashirskoe Shosse, Moscow 115522

tvbek@rambler.ruКоноваловР. Н.KonovalovR. N.

125367, Москва, Волоколамское шоссе, 80

80, Volokolamskoe Shosse, Moscow 125367

ФГБНУ Научноисследовательский институт ревматологии им. В.А.НасоновойРоссияV.A.Nasonova Research Institute of RheumatologyRussian FederationФГБНУ Научный центр неврологииРоссияResearch Center of NevrologyRussian Federation201808052018562249252Copyright © Бекетова Т.В., Коновалов Р.Н., 20182018Бекетова Т.В., Коновалов Р.Н.Beketova T.V., Konovalov R.N.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/2532

Синдром Сусака (СС), или ретино-кохлео-церебральная васкулопатия, является крайне редким тяжелым инвалидизирующим заболеванием, в основе которого предполагают аутоиммунные нарушения. СС характеризуется микроангиопатией с триадой поражения головного мозга, внутреннего уха, сетчатки и относится к междисциплинарным проблемам. Ключевое значение для диагностики СС имеют результаты магнитнорезонансной томографии головного мозга, флюоресцентной ангиографии сетчатки, аудиометрии и взаимодействие врачей различных специальностей – ревматологов, неврологов, офтальмологов, отоларингологов. Представлены описание случая СС и обзор литературы с акцентом на опубликованные в 2018 г. пилотные рекомендации по лечению СС, предполагающие сочетанное применение высоких доз глюкокортикоидов, внутривенного иммуноглобулина, цитостатиков и ритуксимаба.

Susac's syndrome (SS), or retinocochleocerebral vasculopathy, is an extremely rare severe incapacitating disease, the basis for which is assumed to be autoimmune disorders. SS is characterized by microangiopathy with a triad of lesions of the brain, inner ear, and retina and refers to interdisciplinary problems. The results of brain magnetic resonance imaging, retinal fluorescein angiography, and audiometry and the interaction of physicians of various specialties, such as rheumatologists, neurologists, ophthalmologists, and otolaryngologists, are of key importance for the diagnosis of SS. The paper describes a case of SS and reviews the literature with emphasis on pilot recommendations for the treatment of this condition, which have been published in 2018, suggesting the combined use of high-dose glucocorticoids, intravenous immunoglobulin, cytostatic agents, and rituximab.

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