PurposeTo analyze the effect of tumor necrosis factor inhibitor therapy on ocular relapses in patients with Susac syndrome.MethodsMulticenter 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.ResultsFive 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.ConclusionsAnti‐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.
Purpose: To evaluate the effect of tumor necrosis factor (TNF) inhibitor therapy on ocular relapses in patients with Susac syndrome. Methods: Multicenter retrospective cohort study of patients diagnosed with Susac syndrome according to classical clinical criteria. We evaluated the disease activity before and after introduction of anti-TNF therapy and its value as a steroid-sparing agent. Results: Five patients were included. All were initially treated with a combination of corticosteroids and classical immunosuppressive drugs. Infliximab was started in three patients, and adalimumab was started in two patients. Patients had on average 5 ocular relapses during a mean follow-up time of 2.59 years before introducing a TNF inhibitor, corresponding with on average 1.93 relapses per year. After the introduction of an anti-TNF agent, this number was reduced by factor 5.51 to an average of 0.35 relapses per year for a mean follow-up of 2.86 years (P = 0.10). Before anti-TNF introduction ocular relapses occurred at a mean daily dose of 34 mg of prednisone, whereas with anti-TNF treatment, corticosteroid administration could be completely stopped in four patients with one patient still needing 5 mg daily (P = 0.10). Infliximab and adalimumab generally were well tolerated, and no serious adverse events were reported. Conclusion: Although not statistically significant, our results suggest that anti-TNF therapy can be a valuable option for the treatment of ocular Susac syndrome and may especially be considered in those patients unresponsive to more conventional immunosuppressive treatment.
Background and purposeSerum levels of neurofilament light chain (sNfL) and glial fibrillary acidic protein (sGFAP) are promising neuro‐axonal damage and astrocytic activation biomarkers. Susac syndrome (SS) is an increasingly recognized neurological condition and biomarkers that can help assess and monitor disease evolution are highly needed for the adequate management of these patients. sNfL and sGFAP levels were evaluated in patients with SS and their clinical relevance in the relapse and remission phase of the disease was assessed.MethodsAs part of a multicentre study that enrolled patients diagnosed with SS from six international centres, sNfL and sGFAP levels were assessed in 22 SS patients (nine during a relapse and 13 in remission) and 59 age‐ and sex‐matched healthy controls using SimoaTM assay Neurology 2‐Plex B Kit.ResultsSerum NfL levels were higher than those of healthy controls (p < 0.001) in SS patients and in both subgroups of patients in relapse and in remission (p < 0.001 for both), with significantly higher levels in relapse than in remission (p = 0.008). sNfL levels showed a negative correlation with time from the last relapse (r = −0.663; p = 0.001). sGFAP levels were slightly higher in the whole group of patients than in healthy controls (p = 0.046) and were more pronounced in relapse than in remission (p = 0.013).ConclusionIn SS patients, both sNFL and sGFAP levels increased compared with healthy controls. Both biomarkers had higher levels during clinical relapse and much lower levels in remission. sNFL was shown to be time sensitive to clinical changes and can be useful to monitor neuro‐axonal damage in SS.
Susac syndrome should be considered in the differential diagnosis when facing (young) patients with central retinal artery occlusion, especially in the presence of unexplained encephalopathy and/or sensorineural hearing loss.
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