Tocilizumab resolves refractory macular edema associated to retinitis pigmentosa.

Abstract

The aim of this report is to describe the resolution of refractory cystoid macular edema (CME) associated to retinitis pigmentosa (RP) with IV tocilizumab in three patients.Methods: Retrospective study of a series of consecutive cases of patients treated with off-label IV tocilizumab (anti IL6) for CME refractory to acetazolamide 250 mg for 3 months. Patients were diagnosed with RP by fundus appearance, electrophysiology, visual fields, and genetic testing. A complete ophthalmic examination including spectral-domain optical coherence tomography was performed.Results: Three patients with RP and CME refractory to acetazolamide 250 mg for 3 months were treated with monthly IV tocilizumab for at least six months. All patients resolved CME and improved visual acuity after the third month of IV tocilizumab, resolving systemic and ocular adverse events related to previous treatments for CME. Tocilizumab was well tolerated with no other adverse events.Discussion: CME causes visual impairment in RP, but current treatments are usually deficient. Tocilizumab has been successfully used as treatment for refractory CME in uveitis, retinal dystrophies, and autoimmune retinopathies. This article reports, for the first time, the long-term resolution of refractory CME in RP with IV tocilizumab.

Tocilizumab for cystoid macular edema secondary to retinitis pigmentosa

“…IL-6 blockade with TCZ is effective in controlling ME of various etiologies like UME, PME, and ME secondary to npAIR. 4,5,[7][8][9][10] In this short cases series, we demonstrate the excellent effect of TCZ on RP-related CME and RVL. These data are consistent with the recent case series described by Méndez-Martínez et al 10 This is also in keeping with the nding that IL-6 plays a critical role in the genesis of ME, 7 and that IL-6, alongside several other pro-in ammatory cytokines, is up-regulated in patients with RP.…”

“…4,5,[7][8][9][10] In this short cases series, we demonstrate the excellent effect of TCZ on RP-related CME and RVL. These data are consistent with the recent case series described by Méndez-Martínez et al 10 This is also in keeping with the nding that IL-6 plays a critical role in the genesis of ME, 7 and that IL-6, alongside several other pro-in ammatory cytokines, is up-regulated in patients with RP. 11 Therefore, we can reasonably assume that IL-6 pathway activation is a mechanistic component of RP-related CME and RVL.…”

“…[7][8][9] There has been a single other report on intravenous (IV) TCZ administration for retinitis pigmentosa (RP)-related cystoid macular edema (CME). 10 We herein report the second series of patients treated with IV TCZ for RP-related CME and RVL.…”