Age-Related Macular Degeneration Masquerade: A Review of Pentosan Polysulfate Maculopathy and Implications for Clinical Practice

Chirantan Mukhopadhyay; Timothy M Boyce; Karen M Gehrs; James C Folk; Robert F Mullins; Yi Luo; Karl Kreder; Elliott H Sohn

doi:10.1097/APO.0000000000000504

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Age-Related Macular Degeneration Masquerade: A Review of Pentosan Polysulfate Maculopathy and Implications for Clinical Practice

Journal article

Open access

Peer reviewed

Age-Related Macular Degeneration Masquerade: A Review of Pentosan Polysulfate Maculopathy and Implications for Clinical Practice

Chirantan Mukhopadhyay, Timothy M Boyce, Karen M Gehrs, James C Folk, Robert F Mullins, Yi Luo, Karl Kreder and Elliott H Sohn

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.), Vol.11(2), pp.100-110

03/2022

DOI: 10.1097/APO.0000000000000504

PMCID: PMC9096915

PMID: 35533330

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Abstract

Pentosan polysulfate (PPS) sodium (Elmiron) is the only Food and Drug Administration (FDA)-approved oral medication to treat interstitial cystitis, also known as bladder pain syndrome. A symptomatic pigmentary maculopathy associated with PPS was reported in 2018. Since then, recognition of this unique drug toxicity has increased rapidly. This potentially sight-threatening side effect prompted the FDA in June 2020 to update the label for PPS to warn about "retinal pigmentary changes." A challenging feature of pentosan maculopathy is its ability to mimic many other retinal conditions, including inherited retinal dystrophies such as pattern dystrophy, mitochondrially inherited diabetes and deafness, and Stargardt disease, and age-related macular degeneration. In this review, we discuss the history of PPS maculopathy and its implications for thousands of at-risk interstitial cystitis patients. We use published literature and an illustrative case from our institution to highlight the importance of diagnosing PPS maculopathy. We also compare PPS maculopathy to age-related macular degeneration, explain why differentiating between the 2 is clinically important, and highlight avenues for further research. Finally, we highlight the paucity of data on patients of color and why this lack of understanding may impact patient care.

Anticoagulants - adverse effects

Cystitis, Interstitial - chemically induced

Cystitis, Interstitial - diagnosis

Cystitis, Interstitial - drug therapy

Female

Humans

Macular Degeneration - chemically induced

Macular Degeneration - diagnosis

Macular Degeneration - drug therapy

Male

Pentosan Sulfuric Polyester - adverse effects

Retinal Dystrophies

Details

Title: Subtitle: Age-Related Macular Degeneration Masquerade: A Review of Pentosan Polysulfate Maculopathy and Implications for Clinical Practice
Creators: Chirantan Mukhopadhyay - Roy J. and Lucille A. Carver College of Medicine
Timothy M Boyce - University of Iowa
Karen M Gehrs - University of Iowa
James C Folk - University of Iowa
Robert F Mullins - University of Iowa
Yi Luo - University of Iowa
Karl Kreder - University of Iowa
Elliott H Sohn - University of Iowa
Resource Type: Journal article
Publication Details: Asia-Pacific journal of ophthalmology (Philadelphia, Pa.), Vol.11(2), pp.100-110
DOI: 10.1097/APO.0000000000000504
PMID: 35533330
PMCID: PMC9096915
NLM abbreviation: Asia Pac J Ophthalmol (Phila)
ISSN: 2162-0989
eISSN: 2162-0989
Language: English
Date published: 03/2022
Academic Unit: Obstetrics and Gynecology; Fraternal Order of Eagles Diabetes Research Center; Urology; Ophthalmology and Visual Sciences
Record Identifier: 9984321435102771

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