EVALUATION OF TUMOR NECROSIS FACTOR INHIBITOR THERAPY IN SUSAC SYNDROME. Issue 3 (March 2020)

• Record Type: Journal Article
• Authors: Buelens, Tom; Ossewaarde-van Norel, Jeannette; de Boer, Joke H.; Nubourgh, Isabelle; Glibert, Gérald; Kamgang Semeu, Prochore; Fils, Jean-François; Caspers, Laure; Postelmans, Laurence; Willermain, François
• Is Part Of: Retina. Volume 40:Issue 3(2020)
• Publication Date: 2020-03
• Subjects: retinal artery occlusion -- Susac syndrome -- tumor necrosis factor; Retina -- Diseases -- Periodicals; Retinal Diseases; Vitreous Body; 617.735
• DOI: 10.1097/IAE.0000000000002466
• Languages: English
• ISSNs: 0275-004X

Abstract

Abstract : 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. Abstract : Evaluation of the effect of tumor necrosis factor inhibitor therapy on ocular relapses in patients with Susac syndrome and its potential as a steroid-sparing agent.