Treatment
Treatment approaches for Susac syndrome are informed by the severity of CNS involvement, which is defined by clinical features, brain MRI findings, cerebrospinal fluid findings and response to treatment. Treatment is based on expert opinion due to a lack of randomized controlled trial data.8,10
“Early aggressive intensive immunosuppressive treatment is the cornerstone,” said Dr. Furer, “and is associated with better outcomes based on longitudinal follow-up of a cohort of patients with Susac syndrome.”
The combination of immunosuppression is dictated by the severity of CNS involvement. Treatment duration depends on clinical course and response to treatment, but typically lasts at least two years.
Key Takeaways
Dr. Furer wrapped up her talk with the most salient points for a practicing rheumatologist to know:
- Susac symdrome is an autoimmune endotheliopathy targeting the brain, retina and cochlea. CNS involvement is the most common presenting symptom;
- Brain MRI, fluorescein angiography and audiogram are required for diagnosis in all patients suspected of having Susac syndrome;
- Treatment approaches depend on CNS disease severity. Early aggressive immunosuppressive therapy is paramount in severe cases, followed by a slow taper; and
- Multi-disciplinary collaboration is required for optimal management.
Samantha C. Shapiro, MD, is the executive editor of Harrison’s Principles of Internal Medicine. As a clinician educator, she practices telerheumatology and writes for both medical and lay audiences.
References
- Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology. 1979 Mar;29(3):313–316.
- Marrodan M, Fiol MP, Correale J. Susac syndrome: Challenges in the diagnosis and treatment. Brain. 2022 Apr 29;145(3):858–871.
- Peyvandi A, Naghibzadeh B, Roozbahany NA. Neuro-otologic manifestations of multiple sclerosis. Arch Iran Med. 2010 May;13(3):188–192.
- Tugizova M, Feng H, Tomczak A, et al. Case series: Hearing loss in neuromyelitis optica spectrum disorders. Mult Scler Relat Disord. 2020 Jun:41:102032.
- Kastanioudakis I, Ziavra N, Voulgari PV, et al. Ear involvement in systemic lupus erythematosus patients: A comparative study. J Laryngol Otol. 2002 Feb;116(2):103–107.
- Webb CJ, Moots RJ, Swift AC. Ear, nose and throat manifestations of Behçet’s disease: A review. J Laryngol Otol. 2008 Dec;122(12):1279–1283.
- Cereceda-Monteoliva N, Rouhani MJ, Maughan EF, et al. Sarcoidosis of the ear, nose and throat: A review of the literature. Clin Otolaryngol. 2021 Sep;46(5):935–940.
- Rennebohm RM, Asdaghi N, Srivastava S, et al. Guidelines for treatment of Susac syndrome—an update. Int J Stroke. 2020 Jul;15(5):484-494.
- Kleffner I, Dörr J, Ringelstein M, et al. Diagnostic criteria for Susac syndrome. J Neurol Neurosurg Psychiatry. 2016 Dec;87(12):1287–1295.
- Bose S, Papathanasiou A, Karkhanis S, et al. Susac syndrome: Neurological update (clinical features, long-term observational follow-up and management of sixteen patients). J Neurol. 2023 Dec;270(12):6193–6206.
