Journal of neuroimmunology · 2026
Retrospective treatment comparisons in patients with Susac syndrome.
СтатьяСравнительное исследованиеНазвание и аннотация: Перевод в очереди
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Wurth Sebastian, Eberhard Katharina, Sommer Michael, Weger Martin, Enzinger Christian, Di Pauli Franziska, Rauschka Helmut, Seifert-Held Thomas
Аннотация
INTRODUCTION: Susac syndrome is a rare disease affecting arterioles in the brain, retina and inner ear. There is uncertainty about a clinical benefit of adding intravenous immunoglobulins or immunosuppressants to corticosteroid maintenance treatment.
METHODS: This restrospective study identified adult patients from case records who met crite-ria for probable or definite Susac syndrome. Patients with a sole manifestation of arterial wall hyperintensity (AWH) on retinal fluorescein angiography (RFA) were also included. All clinically symptomatic disease activity and clinically asymptomatic AWH on RFA within a 3-month period from first signs were summarized as one event. Following an event, immunosuppressive treatments in consecutive 3-month observation periods were recorded until the next event has occurred or the treatment was discontinued.
RESULTS: Sixteen patients (9 female) were identified (mean age 34 years, range 2065 years). Median follow-up was 79 months (range 16-274 months). In a total of 69 observation periods in all patients, corticosteroids, IVIG, or both in combination, were applied with or without other immunosuppressants. Fewer events occurred in patients treated with IVIG (n = 6) compared to those treated with corticosteroids (n = 10) without statistical significance. Based on our data, a total of 126 observation periods would be required to obtain a statistically significant difference between both treatments, IVIG and corticosteroids. This corresponds to a total of 31.5 patient years needed for a prospective clinical trial.
DISCUSSION: This retrospective analysis in patients with Susac syndrome provides a basis for future structured prospective studies. From our data, a prospective evaluation of an additional clinical benefit of IVIG maintenance therapy in relapse prevention is supported.
METHODS: This restrospective study identified adult patients from case records who met crite-ria for probable or definite Susac syndrome. Patients with a sole manifestation of arterial wall hyperintensity (AWH) on retinal fluorescein angiography (RFA) were also included. All clinically symptomatic disease activity and clinically asymptomatic AWH on RFA within a 3-month period from first signs were summarized as one event. Following an event, immunosuppressive treatments in consecutive 3-month observation periods were recorded until the next event has occurred or the treatment was discontinued.
RESULTS: Sixteen patients (9 female) were identified (mean age 34 years, range 2065 years). Median follow-up was 79 months (range 16-274 months). In a total of 69 observation periods in all patients, corticosteroids, IVIG, or both in combination, were applied with or without other immunosuppressants. Fewer events occurred in patients treated with IVIG (n = 6) compared to those treated with corticosteroids (n = 10) without statistical significance. Based on our data, a total of 126 observation periods would be required to obtain a statistically significant difference between both treatments, IVIG and corticosteroids. This corresponds to a total of 31.5 patient years needed for a prospective clinical trial.
DISCUSSION: This retrospective analysis in patients with Susac syndrome provides a basis for future structured prospective studies. From our data, a prospective evaluation of an additional clinical benefit of IVIG maintenance therapy in relapse prevention is supported.