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

Ophthalmic Surg Lasers Imaging Retina. 2023 Jul;54(7):388-394. doi: 10.3928/23258160-20230522-02. Epub 2023 Jun 1.

Abstract

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; P < 0.0001), and total PPS duration was 12.1 ± 7.1 years (16.0.2 ± 6.1 vs 10.1 ± 6.9; P < 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 mm2 in the PPS-retinopathy group, worsening at a rate of 6.10 ± 10 mm2/year. Patients who developed choroidal neovascular membranes (CNVMs) had faster rates of retinopathy progression (11.6 ± 12 vs 3.53 ± 7.6 mm2/year, P = 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. [Ophthalmic Surg Lasers Imaging Retina 2023;54:388-394.].

MeSH terms

  • Follow-Up Studies
  • Humans
  • Pentosan Sulfuric Polyester / adverse effects
  • Retinal Diseases* / drug therapy
  • Retinitis Pigmentosa*
  • Retrospective Studies
  • Sodium

Substances

  • Pentosan Sulfuric Polyester
  • Sodium