Immunosuppressive and immunomodulation therapies are highly effective in controlling Susac syndrome, a study published in Eye shows. The research also identifies retinal microaneurysms as a new symptom of Susac syndrome

Susac syndrome is a rare autoimmune vasculo-occlusive disease in which the vascular endothelium is damaged by antibodies, resulting in arterial microinfarcts affecting the central nervous system, retina, and inner ear. However, according to researchers, its clinical manifestations can vary and may resemble other neurological, ophthalmic, and auditory diseases.

In this retrospective tertiary center study, investigators assessed imaging, clinical findings, systemic manifestations, and disease outcomes in patients with Susac syndrome on immunosuppressive/immunomodulation therapies. The 7 patients (14 eyes) in the study had a mean age of 34.1 years and were diagnosed at least 12 months prior. Mean follow-up was 31.9 months. All patients had bilateral ocular disease. Researchers reviewed patients’ medical records, including ocular, neurological and auditory clinical and imaging findings, and treatment modalities. 


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The retinal microaneurysms were demonstrated in 5 patients and persisted at the final visit. In 5 eyes, they further extended during follow-up. All patients were treated with immunosuppressive drugs and 5 of 7 received additional immunomodulation therapy. At the last examination, the best-corrected visual acuity was better than 20/40 in all eyes. According to investigators, 1 of 10 eyes had visual field deterioration, no eyes had active ocular disease, all patients achieved neurological stability, and 1 patient had auditory deterioration.

“We believe that ophthalmologists should be aware of this finding when considering the diagnosis of Susac syndrome,” according to the study. “In addition, our study reinforces the importance of a good multidisciplinary collaboration as part of the disease workup and treatment, as Susac syndrome may lead to severe visual, cognitive and auditory impairment while prompt immunosuppressive/immunomodulation therapy targeted according to the clinical findings may be associated with a favorable outcome and achievement of disease stability.”

The authors noted some limitations to their study, including its retrospective design and lack of a visual field test for some eyes. 

Reference

Zur D, Goldstein M, Barequet D, et al. Susac’s syndrome – A new ocular finding and disease outcome. Eye (Lond). April 20, 2021. doi:10.1038/s41433-021-01464-7.