Susac syndrome requires sustained, long term immunotherapy

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Most patients with Susac syndrome, a rare autoimmune condition that can affect the blood vessels in the retina leading to vision loss, require sustained therapy of multiple immune-suppressive, according to data presented at the 2020 AAO Virtual Meeting.

In addition to vision loss, Susac syndrome is characterized by brain disease and hearing loss.

In this retrospective review, 22 patients with Susac syndrome who had at least 12 months of follow-up were included.

Artery occlusions and segmental vascular hyperflourescence were identified by fundus exam in 15 and 11 patients, respectively.

Twelve patients underwent treatment including intravenous immunoglobulin (IVIG) and an antimetabolite, whereas 10 patients with severe disease underwent treatment including IVIG, an antimetabolite, or cyclophosphamide and rituximab.

During the follow-up period, 13 patients experienced flares with a total of 28 episodes, 11 of which were CNS related and 17 retina related.

Patients who were able to successfully stop taking immunotherapy included 6 with milder disease and 1 with severe disease.


Arepalli S, et al. Long-Term Cerebral and Retinal Outcomes of Immunosuppression in Susac Syndrome. Presented at: 2020 AAO Virtual.

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