Evolution of Pentosan Polysulfate Maculopathy Following Medication Discontinuation

Abstract

Purpose: To describe a patient receiving pentosan polysulfate (PPS) for interstitial cystitis, who developed PPS maculopathy (PPSM) that persisted and evolved for four years after medication discontinuation. Methods: Case report of one patient at a tertiary retina clinic. Results: A 48-year-old female with a history of interstitial cystitis was referred to our retina clinic with no vision complaints. She had been taking PPS for 10 years, first consuming 600mg/day for eight years, followed by 200mg/day for two years. At first presentation in 2016, examination revealed macular pigmentary clumping in both eyes. In subsequent years, pigmentary changes were confirmed by optical coherence tomography (OCT) which showed progressive macular thinning in both eyes. In 2019, the patient discontinued PPS; however, she continued to exhibit PPSM progression for years thereafter. At four-year follow-up after discontinuation of PPS, the patient returned with stable vision, but showed changes in macular lipofuscin deposits with outer retinal and retinal pigment epithelial alterations. Conclusion: This case demonstrates sustained and evolving PPSM even after drug discontinuation, suggesting that active PPS may have a prolonged half-life in the retina and retinal pigment epithelium.