Treatment of Susac's syndrome.
Susac's syndrome (SS) is an immune-mediated endotheliopathy that affects the microvasculature of the brain, retina, and inner ear. SS responds well to immunosuppressive therapies when treatment is prompt, aggressive, and sustained. Striking similarities exist between SS and dermatomyositis (DM), regarding immunopathogenesis, natural history, and treatment needs. We apply lessons learned from study of DM to SS, and offer our current treatment protocol for SS. Since these treatment guidelines are based mainly on anecdotal evidence, they represent only preliminary recommendations.
Rennebohm
Robert M
RM
Clinical Pediatrics, Division of Pediatric Rheumatology, Ohio State University, College of Medicine and Public Health, Columbus Children's Hospital, 700 Children's Drive, Columbus, OH 43205, United States. rennebohmr@pediatrics.ohio-state.edu
Susac
John O
JO
D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology* / Q000503:physiopathology; D001927:Brain Diseases / Q000188:drug therapy* / Q000276:immunology* / Q000503:physiopathology; D002561:Cerebrovascular Disorders / Q000188:drug therapy* / Q000276:immunology* / Q000503:physiopathology; D042783:Endothelial Cells / Q000276:immunology / Q000473:pathology; D034381:Hearing Loss / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D002537:Intracranial Arteriosclerosis / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D008833:Microcirculation / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D015356:Retinal Artery Occlusion / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D013577:Syndrome
D007166:Immunosuppressive Agents