Published online

Journal Article

PURPOSE: To demonstrate a rapid improvement of recalcitrant cystoid macular edema (CME) and perivascular leakage, in a patient with non-paraneoplastic autoimmune retinopathy and autoimmune optic neuropathy after treatment with sarilumab, a human anti-interleukin-6 (IL-6) receptor antibody. METHODS: Observational case report. RESULTS: A 29-year-old woman was diagnosed with non-paraneoplastic autoimmune retinopathy and autoimmune optic neuropathy and followed over 1.5 years. She had recalcitrant CME despite local corticosteroid and immunosuppressive therapy that included azathioprine and adalimumab. Subcutaneous sarilumab was initiated at a dose of 200 mg every 2 weeks. Cystoid macular edema significantly decreased after two injections and resolved after four injections with associated improvement in visual acuity and significant improvement in perivascular leakage on fluorescein angiography. There was a sustained visual and anatomical improvement at 6 months along with mild improvement in electroretinogram responses. The patient tolerated the medication with no side effects. CONCLUSION: Management of CME in non-paraneoplastic autoimmune retinopathy is challenging, and long-term immunosuppression is often employed with varying degrees of success. The improvement in refractory CME and perivascular leakage in this case supports the potential role of an IL-6 inhibitor to treat CME associated with non-paraneoplastic autoimmune retinopathy suggesting the role.

Full Text

Duke Authors

Cited Authors

  • Grewal, DS; Jaffe, GJ; Keenan, RT

Published Date

  • April 8, 2019

Published In

PubMed ID

  • 30986811

Pubmed Central ID

  • 30986811

Electronic International Standard Serial Number (EISSN)

  • 1937-1578

Digital Object Identifier (DOI)

  • 10.1097/ICB.0000000000000872


  • eng

Conference Location

  • United States