Reduction in Cystoid Macular Edema and Ellipsoid Zone Reconstitution in Non-paraneoplastic Autoimmune Retinopathy Treated with Anti-Interleukin-6 Monoclonal Antibodies

Abstract

Purpose : To report the effect of anti-interleukin-6 (IL-6) receptor monoclonal antibodies tocilizumab and sarilumab eyes with non-paraneoplastic autoimmune retinopathy (npAIR) with cystoid macular edema (CME)

Methods : Retrospective case series including 14 eyes of 8 patients with npAIR and CME treated with anti-IL-6 medications.Visual acuity (VA) and central subfield thickness (CST), total macular volume (TMV), and ellipsoid zone integrity change as measured optical coherence tomography were extracted from charts prior to and at 3, 6, 12, 18, and 24 months after anti-IL-6 therapy was initiated. Eyes that had a >20% reduction in CST were defined as treatment responders, >20% increase in CST as failures, and ≤20% change in CST as stable.

Results : Eleven eyes of six patients had failed multiple prior immunosuppressive therapies. There was a significant reduction (P<.05) in CST baseline (473.1 μm) after initiation of anti-IL-6 therapy that began at 3 months (389.2 μm) and continued through 24 months (348.7 μm). Similarly, there was a significant reduction (P<.05) in TMV baseline (10.15 μm³) after initiation of anti-IL-6 therapy that began at 3 months (8.91 μm³) and continued through 24 months (7.67 μm³). VA improved from 0.84 to 0.52 at 24-month follow-up, a change that did not reach statistical significance (P=.157). Nine of 14 eyes (64.3%) were treatment responders, 4 eyes (28.6%) were stable, and 1 eye (7.1%) was a treatment failure. See Table 1 for full results. Prior to starting anti-IL-6 therapy, 9 of 14 eyes (64.3 %) of eyes had EZ integrity loss which was evident on macular OCT with an average loss of 30.0 μm per month. After starting IL-6 therapy, there was a significant decrease in rate of EZ change, experiencing an average restoration of 22.7 μm per month (P=.008). It was possible to discontinue rituximab in all 4 patients who were on this therapy for AIR, and at least one eye of all patients responded to anti-IL-6 treatment. No patient had adverse events or required discontinuation of therapy while on anti-IL-6 medication.

Conclusions : In this cohort of patients with npAIR and CME, treatment with anti-IL-6 medications tocilizumab and sarilumab, was associated with reduced CME, partial restoration of the ellipsoid zone, and a trend towards improved VA, while reducing burden of prior immunosuppressive therapies.

This is a 2021 ARVO Annual Meeting abstract.