Evaluation of Tumor Necrosis Factor inhibitor therapy in Susac syndrome

Purpose To analyze the effect of tumor necrosis factor inhibitor therapy on ocular relapses in patients with Susac syndrome. Methods Multicenter retrospective case series of patients with ocular Susac syndrome treated with a TNF inhibitor (either infliximab or adalimumab). Diagnosis was based on neurologic, ophthalmic, otologic, biologic and imaging typical findings. Results Five patients were included. All were initially treated with a combination of corticosteroids and classical immunosuppressive drugs. In 4 of the 5 patients a TNF‐inhibitor (3 infliximab and 2 adalimumab) was started due to treatment failure. In these patients cortisone could not be decreased below a daily dose of 10 mg without the occurrence of relapses (with an average of 4.25 relapses over a mean follow‐up of 330 days) despite the use of a mean of 2.75 different immunosuppressive drugs. After introduction of an anti‐TNF agent the daily cortisone dose could successfully be tapered below 10 mg in all patients with a complete stop in 3 patients, with a mean number of 1.25 relapses during a mean follow‐up of 1,199 days. In the fifth patient, disease activity was well controlled with only steroid sparing immunosuppressive agents and only 1 relapse during approximately 5 years of follow up until these had to be stopped for a desired pregnancy. No relapse was documented during 60 weeks of follow‐up while on infliximab treatment afterwards. Conclusions Anti‐TNF antibodies can be a valuable option for the treatment of ocular Susac syndrome and may especially be considered in those patients unresponsive to conventional immunosuppressive treatment.