Journal article
Expanded Clinical Spectrum of Pentosan Polysulfate Maculopathy: A Macula Society Collaborative Study
Ophthalmology retina, Vol.6(3), pp.219-227
07/2021
DOI: 10.1016/j.oret.2021.07.004
Abstract
Explore the spectrum of clinical manifestations of pentosan polysulfate sodium (PPS) maculopathy observed across a range of practice settings.
Multi-institutional retrospective study.
Patients exhibiting findings suggestive of PPS maculopathy identified from April 30, 2019, to December 4, 2020.
Members of the Macula Society submitted cases of presumed PPS maculopathy for consideration in this series. Diagnosis was confirmed by masked review of fundus imaging. Clinical characteristics of confirmed cases were summarized with descriptive statistics.
Pentosan polysulfate exposure characteristics and fundus imaging features.
There were 74 patients with PPS maculopathy included in the current study. Median (interquartile range) age at diagnosis was 62.0 years (56.0–65.8). The median duration of exposure to PPS was 14.0 years (10.2–18.9), with a median cumulative exposure of 1.5 kg (0.9–2.4). The most common presenting symptom was decreased or blurry vision (66.2%), followed by prolonged dark adaption or nyctalopia (32.4%). The most common referral diagnosis was age-related macular degeneration (54.1%); 16.2% of patients were referred for suspected PPS maculopathy. Novel imaging findings emerged, including highly asymmetric disease in 2 patients and a prominent vitelliform maculopathy in 2 patients.
Most patients with PPS maculopathy exhibit characteristic findings on multimodal fundus imaging in the setting of high cumulative exposure to the oral drug. Some patients in the current study manifested novel imaging findings, expanding our understanding of the phenotypic spectrum of this condition. We recommend considering standardized ophthalmic screening of patients treated with PPS.
Details
- Title: Subtitle
- Expanded Clinical Spectrum of Pentosan Polysulfate Maculopathy: A Macula Society Collaborative Study
- Creators
- Nieraj Jain - Emory UniversityAlbert Liao - Emory UniversitySunir J Garg - Wills Eye HospitalSamir N Patel - Wills Eye HospitalCharles C Wykoff - Houston MethodistHannah J Yu - Houston MethodistNikolas J.S London - Retina Consultants San Diego, Poway, CaliforniaRahul N Khurana - University of California, San FranciscoDavid N Zacks - University of Michigan–Ann ArborAudina M Berrocal - Bascom Palmer Eye InstituteBrandon J Lujan - Oregon Health & Science UniversityTyler E Greenlee - Cleveland ClinicRishi P Singh - Cleveland ClinicAndrew Hendrick - Emory UniversityBenjamin I Meyer - Emory UniversityGhazala A O’KeefeRachel Shah - Emory UniversityJiong Yan - Emory UniversityMina M Chung - University of RochesterAllen Chiang - Wills Eye InstituteJames P Dunn - Wills Eye InstituteMitchell S Fineman - Wills Eye InstituteDavid H Fischer - Wills Eye InstituteOmesh P Gupta - Wills Eye InstituteAllen C Ho - Wills Eye InstituteJason Hsu - Wills Eye InstituteThomas L Jenkins - Wills Eye InstituteRichard S Kaiser - Wills Eye InstituteCarl H Park - Wills Eye InstituteArunan Sivalingam - Wills Eye InstituteMarc J Spirn - Wills Eye InstituteJames F Vander - Wills Eye InstituteYoshihiro Yonekawa - Wills Eye InstituteGregg T KokameMaria WonDavid M BrownAmy C ScheflerLawrence S HalperinAla Moshiri - University of California, DavisSusanna S Park - University of California, DavisJames C Folk - University of IowaElliott H Sohn - University of IowaAlexis K Warren - University of IowaLinda A Lam - University of Southern CaliforniaH. Richard McDonaldCaleb C NgMichelle Y PengPeter J BelinRobert A MittraK. Bailey FreundKapil Mishra - Johns Hopkins UniversityMandeep S Singh - Johns Hopkins UniversityMacula Society Pentosan Polysulfate Maculopathy Study Group
- Resource Type
- Journal article
- Publication Details
- Ophthalmology retina, Vol.6(3), pp.219-227
- Publisher
- Elsevier Inc
- DOI
- 10.1016/j.oret.2021.07.004
- ISSN
- 2468-6530
- eISSN
- 2468-6530
- Grant note
- DOI: 10.13039/501100000262, name: Foundation Fighting Blindness; DOI: 10.13039/100001116, name: Foundation Fighting Blindness, award: CD-C-0918-0748-EEC
- Language
- English
- Date published
- 07/2021
- Academic Unit
- Ophthalmology and Visual Sciences; Fraternal Order of Eagles Diabetes Research Center
- Record Identifier
- 9984187015102771
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