Progression of Pentosan-Polysulfate Sodium-Associated Retinopathy: Intermediate Follow-Up.

Background And Objective: To compare the risk factors for the development and progression of pigmentary retinopathy in patients exposed to pentosan polysulfate sodium (PPS).

Materials And Methods: Retrospective cohort study of patients exposed to PPS with at least two follow-up visits with multimodal imaging.

Results: A total of 97 patients were included (33 with PPS-associated retinopathy and 64 without). The average follow-up was 29.4 months, overall cumulative dose was 1,220 ± 910 g (1,730 ± 870 vs 959 ± 910; < 0.0001), and total PPS duration was 12.1 ± 7.1 years (16.0.2 ± 6.1 vs 10.1 ± 6.9; < 0.0001). The best-corrected visual acuity remained stable during follow-up. At presentation, the average area of the retinopathy in the worse eye was 54.1 ± 50 mm in the PPS-retinopathy group, worsening at a rate of 6.10 ± 10 mm/year. Patients who developed choroidal neovascular membranes (CNVMs) had faster rates of retinopathy progression (11.6 ± 12 vs 3.53 ± 7.6 mm/year, = 0.036). No patient had the exact same gene mutation.

Conclusion: PPS-associated pigmentary retinopathy can continue to progress over time, even after discontinuing the medication. CNVM development may be associated with faster rates of retinopathy progression. .