Pentosan polysulfate sodium maculopathy: Final analysis of a prospective cohort, updated review, and association with inflammatory bowel disease.

Swetha B Velaga, Adrian Au, Muneeswar Nittala, SriniVas Sadda, Alice Wong, Jay Chhablani, David Sarraf, Alessandro Feo, Elodie Bousquet, Ahmad Santina, Néda Abraham, Meira Fogel-Levin, Veronica Romero-Morales, Swathi Somisetty, Anthony Lu, Shilo Voichanski

Journal: Survey of ophthalmology 2025;70(3):583-592

PMID: 39674406

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Abstract

Pentosan polysulfate sodium (PPS) maculopathy is a recently described condition of macular toxicity. We report the prevalence (15 %), the clinical characteristics, and the multimodal imaging (MMI) features of PPS maculopathy based on a final prospective analysis of a cohort of 154 subjects and highlight the association with inflammatory bowel disease (IBD). In addition, we summarize the most recent and relevant literature on PPS maculopathy. This study and other recent analyses provide solid evidence of the toxic effects of PPS on the retina and choroid and refute the concept that the maculopathy is an association of the underlying disease, interstitial cystitis. Moreover, this study and others raise a major concern that PPS may also be toxic to the bowel. Vigilant surveillance of patients receiving this potentially harmful drug is critical. We review the recommended screening guidelines, the pathophysiology and MMI spectrum of disease, the differential diagnosis including MMI clues to an accurate diagnosis, and the progression of retinopathy that can take place despite discontinuation of the drug, emphasizing the critical importance of early detection of toxicity.

Published by Elsevier Inc.

Address: Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, CA, United States.; Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, CA, United States; Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4, Pieve Emanuele-Milan 20072, Italy.; Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, CA, United States; Department of Ophthalmology, University of Paris Cité, Lariboisière Hospital, Assistance Publique-Hôpitaux de Paris, France.; Doheny Eye Institute, University of California, Los Angeles, CA, United States.; Goldschleger Eye Institute, Sheba Medical Center, and Sackler Faculty of Medicine, Tel Aviv University, Israel.; Department of Ophthalmology, UPMC Eye Center, University of Pittsburgh, Pittsburgh, PA, USA.; Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, CA, United States; Vitreoretinal Division, Ophthalmology Department, Shaare Zedek Medical Center, Jerusalem, Israel.; Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, CA, United States; Greater Los Angeles VA Healthcare Center (D.S.), Los Angeles, CA, USA. Electronic address: [email protected].

DOI: 10.1016/j.survophthal.2024.12.002

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MeSH Terms: Humans, Pentosan Sulfuric Polyester, Inflammatory Bowel Diseases, Retinal Diseases, Prospective Studies, Tomography, Optical Coherence, Prevalence, Macula Lutea