SUSAC'S SYNDROME (RETINOCOCHLEOCEREBRAL VASCULOPATHY)
https://doi.org/10.14412/1995-4484-2018-249-252
Abstract
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.
About the Authors
T. V. Beketova
V.A.Nasonova Research Institute of Rheumatology
Russian Federation
Russian Federation
Tatiana Beketova.
34A, Kashirskoe Shosse, Moscow 115522
R. N. Konovalov
Research Center of Nevrology
Russian Federation
Russian Federation
80, Volokolamskoe Shosse, Moscow 125367
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Review
For citations:
Beketova T.V., Konovalov R.N. SUSAC'S SYNDROME (RETINOCOCHLEOCEREBRAL VASCULOPATHY). Rheumatology Science and Practice. 2018;56(2):249-252. (In Russ.) https://doi.org/10.14412/1995-4484-2018-249-252
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