Pentosan Polysulfate Sodium (PPS, Elmiron) Maculopathy Demonstrates the Importance of Coordinated Care from a Low Vision Perspective

Authors

• Charlotte I. Spada OD, University of the Incarnate Word Rosenberg School of OptometryFollow
• Stephanie Schmiedecke Barbieri OD, University of Incarnate Word Rosenberg School of OptometryFollow
• Patricia Sanchez Diaz DVM, PhD, University of the Incarnate Word Rosenberg School of OptometryFollow
• Jeffrey Rabin OD, PhD, University of the Incarnate Word Rosenberg School of OptometryFollow

Medical Subject Headings

Pentosan Polysulfate; Macular Degeneration; Retinal Diseases; Fundus Autofluorescence; Toxicology; Interstitial Cystitis; Vision Disorders; Low Vision; Rehabilitation; Electrodiagnosis; Genetic Testing; Ophthalmology

Abstract

Background: Pentosan polysulfate sodium (PPS, Elmiron®) is a widely used medication to reduce pain caused by interstitial cystitis, a bladder disorder most often affecting females. PPS maculopathy is a novel form of toxic retinal disease often not recognized or mistaken for other retinal conditions such as macular degeneration due to the initial similarities on fundus exam. However, when visualized using fundus autofluorescence, affected eyes show characteristic hyper/hypo-autofluorescent patterns.

Case report: A patient taking PPS for 10 years presented with macular changes and complaints of blurry vision and nyctalopia. Clinical, electrodiagnostic, and genetic testing were performed including fundus autofluorescence and suggested PPS-related maculopathy. Timeline images were also obtained from the patient's referring provider aiding in the diagnosis. We provided our patient with introductory low vision rehabilitative care including separate pairs of reading and distance glasses, increased lighting, and yellow filters for reduced contrast sensitivity. The patient was also referred to a retina specialist for ongoing care. Our patient illustrates the importance of early referrals to low vision as well as the importance of coordinated care.

Conclusion: PPS maculopathy is progressive in nature even after the cessation of medication. Therefore, it is vital for patients to obtain an early diagnosis and to have a team approach to care including management for the systemic and retinal disease, low vision and counseling services, and PCP/urologist, at a minimum.

Recommended Citation

Spada CI, Schmiedecke Barbieri S, Sanchez Diaz P, Rabin J. Pentosan Polysulfate Sodium (PPS, Elmiron) Maculopathy Demonstrates the Importance of Coordinated Care from a Low Vision Perspective. Optometric Clinical Practice. 2026; 8(2):20. https://doi.org/10.37685/uiwlibraries.2575-7717.8.2.1055

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Digital Object Identifier (DOI)

10.37685/uiwlibraries.2575-7717.8.2.1055