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Related Experiment Video

Updated: Nov 5, 2025

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Pentosan polysulfate maculopathy.

Aaron Lindeke-Myers1, Adam M Hanif2, Nieraj Jain3

  • 1Emory University School of Medicine, Atlanta, GA, USA.

Survey of Ophthalmology
|May 17, 2021
PubMed
Summary
This summary is machine-generated.

Pentosan polysulfate sodium (PPS), an interstitial cystitis treatment, is linked to a progressive maculopathy. This vision-threatening condition affects the retinal pigment epithelium and may worsen post-cessation.

Keywords:
Elmirondrug toxicityinterstitial cystitismaculopathymedication reactionpattern dystrophypentosan polysulfate sodiumpigmentary maculopathy

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Area of Science:

  • Ophthalmology
  • Pharmacology
  • Toxicology

Background:

  • Pentosan polysulfate sodium (PPS) is the sole oral FDA-approved therapy for interstitial cystitis.
  • Emerging evidence links long-term PPS use to a progressive, vision-threatening maculopathy.

Purpose of the Study:

  • To review and consolidate clinical features of PPS maculopathy.
  • To evaluate the strength of the association between PPS exposure and maculopathy.

Main Methods:

  • Systematic review of all published literature on PPS maculopathy.
  • Consolidation of clinical features, dose-dependency, and progression patterns.

Main Results:

  • A strong, dose-dependent association exists between PPS use and maculopathy affecting the retinal pigment epithelium (RPE) and RPE-photoreceptor interface.
  • Symptoms include prolonged dark adaptation and reading difficulty; fundus autofluorescence reveals characteristic hypo- and hyperautofluorescent spots and a peripapillary halo.
  • Potential complications include RPE atrophy, cystoid macular edema, and neovascularization, risking central vision loss.

Conclusions:

  • PPS use is associated with a significant public health risk due to progressive maculopathy.
  • Ophthalmologists should screen PPS users with multimodal imaging.
  • Prescribers should minimize PPS dose and duration to mitigate risk.