Treatment outcomes of patients with non-paraneoplastic autoimmune retinopathy

Abstract

Purpose : This retrospective, observational study aimed to assess the efficacy of intravenous immunoglobulin (IVIG) along with IV methylprednisolone (IVMP) therapy ± rituximab ± bortezomib therapies for patients with non-paraneoplastic autoimmune retinopathy (np-AIR).

Methods : Medical records of patients who have been managed in the Uveitis Clinic at a tertiary eye care center between 2017 and 2023 were reviewed. Ten patients (20 eyes) diagnosed with np-AIR and received IVIG along with IVMP therapy ± rituximab ± bortezomib for at least 12 months were included. All patients had progressive loss of vision prior to receiving systemic therapy. Patient’s demographics, ocular findings, clinical data, treatment outcomes using best corrected visual acuity (BCVA), goldman visual field (GVF), and electroretinogram (ERG) were reviewed. Follow-up BCVA, GVF, and ERG results were categorized into improved, stable, or worsened subgroups.

Results : The mean age (5 males and 5 females) was 51.51 ± (24.24) years. The average time to treatment initiation was 29.6 ± (29.5) months. Eight patients were positive for anti-retinal antibodies and six patients were positive for anti-optic nerve antibodies. All 10 patients received IVIG along with IVMP; in addition, 7 of them received rituximab, and 2 of them received bortezomib. The mean follow-up time was 47.7 ± (15.4) months, and mean duration of therapy was 26.3 ± (17.5), 21.2 ± (16), 29.5 ± (19.5), and 12 ± (1.4) months for IVIG, IVMP, rituximab, and bortezomib therapies, respectively. Concomitant immunomodulators/biologics included adalimumab (1 patient), cyclosporine (1 patient), azathioprine (1 patient), and mycophenolate mofetil (2 patients). The mean BCVA at baseline was 0.45 ± (0.56). BCVA was stable/improved in 13 eyes. GVF was stable in 2 eyes and improved in 5 eyes. VEP amplitude values were stable or improved in 12 eyes, full field ERG (ffERG) amplitude values were improved or stable in 13 eyes, ffERG implicit time values were stable or improved in 17 eyes, and multifocal (mfERG) amplitude values were improved or stable in 9 eyes and mfERG implicit time values were improved or stable in 15 eyes.

Conclusions : Majority of patients who received IVIG along with IVMP ± rituximab ± bortezomib demonstrated stable or improved BCVA and ERG. IVIG along with IVMP ± rituximab ± bortezomib therapy may be considered as potentially effective therapeutic option for patients with autoimmune retinopathy.

This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.