Abstract
BackgroundAutoimmune retinopathy (AIR) is a rare but potentially blinding condition that is often underdiagnosed. Common features in AIR presentation include rapidly progressive vision loss with abnormal electrophysiological responses of the retina associated with positive anti-retinal antibodies. AIR is also challenging to treat, and thus, the introduction of new potential therapeutic agents is welcomed. The goal of this communication is to assess the effects of rituximab infusions on electroretinogram (ERG) responses and visual function outcomes in patients with non-paraneoplastic autoimmune retinopathy (npAIR).ResultsFollowing infusion(s), three out of five patients showed no evidence of disease progression or improved, while two patients continued to progress on ERG. One patient demonstrated improvement in visual acuity (2 lines) in both eyes. ERG responses provided objective monitoring of patients’ visual function and response to immunosuppression over time.ConclusionsThese findings suggest that patients with npAIR unresponsive to other immunosuppression therapies may benefit from rituximab infusion, although stabilization rather than improvement was more frequently the outcome in our case series. Furthermore, regularly scheduled ERG follow-up examinations are recommended for monitoring patients’ progression during treatment.
Highlights
Autoimmune retinopathy (AIR) is a rare but potentially blinding condition that is often underdiagnosed
AIR include such conditions as paraneoplastic autoimmune retinopathy, which can be further subdivided into cancerassociated retinopathy (CAR) and melanoma-associated retinopathy (MAR)
Three patients (P1, 2, and 5) had a past medical history of systemic autoimmune disorder, and Patient 4 (P4) was diagnosed with Crohn disease 2 years after the initial non-paraneoplastic autoimmune retinopathy (npAIR) diagnosis (Table 1)
Summary
Autoimmune retinopathy (AIR) is a rare but potentially blinding condition that is often underdiagnosed. AIR is challenging to treat, and the introduction of new potential therapeutic agents is welcomed. The goal of this communication is to assess the effects of rituximab infusions on electroretinogram (ERG) responses and visual function outcomes in patients with non-paraneoplastic autoimmune retinopathy (npAIR). The cell types that are Rituximab is a monoclonal antibody that binds to CD20, a non-glycosylated protein expressed on the surface of B lymphocytes (B-cells), inducing B-cell lysis [10] It was first approved by the FDA for the treatment of B-cell lymphoma, recently it has been applied to a variety of autoimmune disorders [11]. Our aim is to provide a reference for clinicians who are seeking new options for managing this complicated disease and to demonstrate the utility of the ERG as a means of assessing response to immunosuppression in npAIR
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