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Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model

Robert M Rennebohm1 email, Martin Lubow2 email, Jerome Rusin3 email, Lisa Martin3 email, Deborah M Grzybowski2 email and John O Susac4 email

1Department of Pediatrics, Division of Pediatric Rheumatology, Ohio State University College of Medicine, Columbus, Ohio, USA

2Department of Ophthalmology, Ohio State University College of Medicine, Columbus, Ohio, USA

3Department of Radiology, Nationwide Children's Hospital, Columbus, Ohio, USA

4Neurology and Neurosurgery, PA, Winter Haven, FL, USA

author email corresponding author email

Pediatric Rheumatology 2008, 6:3doi:10.1186/1546-0096-6-3

Published: 29 January 2008

Abstract

We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.


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