Pentosan Polysulfate Sodium-Associated Pigmentary Retinopathy: Risk Factors and Fundus Findings

Abstract

Purpose

To evaluate the risk factors and fundus findings of patients with potential PPS-associated retinopathy.

Patients and Methods

A retrospective chart review was performed of patients exposed to PPS who had a dilated fundus examination at a large retina-only practice from 2018–21. Multimodal images were evaluated by masked reviewers.

Results

A total of 148 patients were included, of whom 33 (22%) had PPS-associated retinopathy, and 115 (78%) did not. The mean age was 60.3 years old, and the mean follow-up was 11.8 months. The PPS-associated retinopathy group had higher mean cumulative doses of PPS (1600g±849 vs 864g±852, P < 0.0001, Mann–Whitney test) and longer duration of PPS use (13.6 years vs 7.48, P < 0.0001). There was no statistically significant difference based on a history of kidney or liver disease or the dosage per day for the weight, body mass index, body surface area, or lean body weight. Of the patients with PPS-associated retinopathy whose genetic results were available, 15 of 16 (93%) were heterozygous for variants of uncertain significance.

Conclusion

A longer duration of PPS use and higher cumulative dosage of PPS were associated with an increased risk of developing PPS-associated pigmentary retinopathy. The role of genetic mutations in patients exposed to PPS is still to be determined.

Keywords:

• pentosan polysulfate sodium
• pigmentary retinopathy
• pharmacotoxicity
• inherited retinal dystrophies
• genetics

Acknowledgments

The authors are extremely grateful to the contributions of David Chin Yee MD, Michael Jacobson MD, Morgan Jackson COA, Sean Koh MD, Scott Lampert MD, Mark Rivellese MD, Jay Stallman MD, Robert Stoltz MD PhD, Stephanie Vanderveldt MD, and Harpreet (Paul) Walia MD at Georgia Retina.

Disclosure

The authors report no conflicts of interest in this work.

Additional information

Funding

There is no funding to report.