Susac syndrome: The effectiveness of Rituximab monotherapy
https://doi.org/10.47360/1995-4484-2023-385-388
Abstract
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.
Keywords
Supporting agencies: The study was not funded by sponsorship. The researchers are solely responsible for submitting the final manuscript for publication. The author participated in the conception and design of the study and in the writing of the manuscript. The final version of the manuscript has been approved by the author. The author did not receive a fee for the research.
About the Author
T. V. Beketova
Central State Medical Academy of the Administrative Directorate of the President of the Russian Federation;
V.A. Nasonova Research Institute of Rheumatology;
Moscow Polytechnic University
Russian Federation
Russian Federation
121359, Moscow, Marshala Timoshenko str., 19, building 1A;
115522, Moscow, Kashirskoye Highway, 34A;
107023, Moscow, Bolshaya Semyonovskaya str., 38
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Review
For citations:
Beketova T.V. Susac syndrome: The effectiveness of Rituximab monotherapy. Rheumatology Science and Practice. 2023;61(3):385-388. https://doi.org/10.47360/1995-4484-2023-385-388
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