Ryan D. Salazar, Krystol R. Weidner, Caroline R. Alquist
{"title":"Therapeutic plasma exchange in refractory Susac's syndrome: A brief report","authors":"Ryan D. Salazar, Krystol R. Weidner, Caroline R. Alquist","doi":"10.1002/jca.22098","DOIUrl":null,"url":null,"abstract":"<p>Susac's syndrome (SuS) is an autoimmune endotheliopathy that typically presents with the clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusion. It has a wide range of possible presentations, and its pathogenesis remains uncertain. Fulminant and refractory cases are difficult to treat, and no standard treatment protocol has been established. However, therapeutic plasma exchange (TPE) has been described as an adjunctive therapy in several SuS cases. Herein we present a case of a 63-year-old male with debilitating encephalopathy and recent hearing and vision loss, who responded favorably to TPE. Given this and other published reports of plasma exchange therapy for SuS, treatment protocols should consider TPE in early stages of disease.</p>","PeriodicalId":15390,"journal":{"name":"Journal of Clinical Apheresis","volume":"39 1","pages":""},"PeriodicalIF":1.4000,"publicationDate":"2023-11-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jca.22098","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Clinical Apheresis","FirstCategoryId":"3","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1002/jca.22098","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Susac's syndrome (SuS) is an autoimmune endotheliopathy that typically presents with the clinical triad of encephalopathy, hearing loss, and branch retinal artery occlusion. It has a wide range of possible presentations, and its pathogenesis remains uncertain. Fulminant and refractory cases are difficult to treat, and no standard treatment protocol has been established. However, therapeutic plasma exchange (TPE) has been described as an adjunctive therapy in several SuS cases. Herein we present a case of a 63-year-old male with debilitating encephalopathy and recent hearing and vision loss, who responded favorably to TPE. Given this and other published reports of plasma exchange therapy for SuS, treatment protocols should consider TPE in early stages of disease.
期刊介绍：
The Journal of Clinical Apheresis publishes articles dealing with all aspects of hemapheresis. Articles welcomed for review include those reporting basic research and clinical applications of therapeutic plasma exchange, therapeutic cytapheresis, therapeutic absorption, blood component collection and transfusion, donor recruitment and safety, administration of hemapheresis centers, and innovative applications of hemapheresis technology. Experimental studies, clinical trials, case reports, and concise reviews will be welcomed.
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