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Outcomes of Maintenance Immunotherapy in a Cohort of Patients With Susac Syndrome: A 2-Center Large Case Series
Journal article   Peer reviewed

Outcomes of Maintenance Immunotherapy in a Cohort of Patients With Susac Syndrome: A 2-Center Large Case Series

Negar Moheb, Deena A Tajfirouz, Kevin D Chodnicki, Andrew McKeon, Natthapon Rattanathamsakul, Abdul-Rahman Salman, Leigh A Rettenmaier, Aditi Varma-Doyle, Bart K Chwalisz and John J Chen
Neurology. Clinical practice, Vol.15(6), e200544
12/2025
DOI: 10.1212/CPJ.0000000000200544
PMCID: PMC12507439
PMID: 41070138
url
https://pmc.ncbi.nlm.nih.gov/articles/PMC12507439/View
Open Access

Abstract

Susac syndrome (SuS) is a rare autoimmune disorder characterized by the classic clinical triad of encephalopathy, sensorineural hearing loss, and branch retinal artery occlusion (BRAO). Maintenance immunotherapy is important in most cases to prevent new attacks that can cause significant disability. However, owing to the rarity of SuS, there are few large studies evaluating the efficacy of immunotherapies in SuS. In this large case series, we evaluated the association of utilized immunotherapies and relapse rates in the management of SuS from a large multicenter cohort. Retrospective data analysis of 54 patients with definite or probable SuS diagnoses who were evaluated and managed at 2 academic medical centers in the United States. The percentage with relapse and annualized relapse rate (ARR) were calculated for each immunotherapy. The median age at diagnosis of SuS was 36.5 years (interquartile range 30-46 years), and 61% were female. On maintenance immunotherapy, the relapse rate and median ARR were as follows: mycophenolate mofetil 40% (12 of 30 with a median duration of 1.08 years; ARR 0), low or intermediate dose IV immunoglobulin (IVIG) 42.3% (11 of 26 with a median duration of 0.75 years; ARR 0.1), high dose IVIG 11.7% (2 of 17 with a median duration of 0.58 years; ARR 0), cyclophosphamide 36.3% (8 of 22 with a median duration 0.5 years; ARR 0), rituximab 38% (8 of 21 with a median duration of 1.58 years; ARR 0), azathioprine 60% (3 of 5 with a median duration of 1.33 years; ARR 0.1), methotrexate 66.6% (2 of 3 with a median duration of 5.5 years, ARR 0.1), infliximab 0% (0 of 1 with duration of 0.66 years; ARR 0), maintenance plasma exchange 0% (0 of 1 with duration of 1.25 years; ARR 0), and tocilizumab 0% (0 of 1 with duration of 0.91; ARR 0). Our relatively large cohort of patients with SuS shows a variety of maintenance immunotherapies are used with varying response rates. Among our patients, a number of refractory cases required aggressive and combination of immunotherapies.

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