RETINAL, VITREOUS AND MACULAR DISORDERS: Edited by Brandon G. Busbee and John W. KitchensUpdate on maculopathy secondary to pentosan polysulfate toxicityAbou-Jaoude, Michelle; Fraser, Claire; Maldonado, Ramiro S. Author Information Department of Ophthalmology and Visual Sciences, University of Kentucky, Lexington, Kentucky, USA Correspondence to Ramiro S. Maldonado, MD, 110 Conn Terrace, Suite 550, Department of Ophthalmology, University of Kentucky, Lexington, KY 40508, USA. E-mail: [email protected] Current Opinion in Ophthalmology: May 2021 - Volume 32 - Issue 3 - p 233-239 doi: 10.1097/ICU.0000000000000754 Buy Metrics Abstract Purpose of review The aim of the present review is to provide a comprehensive summary of available knowledge regarding toxic maculopathy secondary to pentosan polysulfate sodium (PPS). Recent findings PPS toxicity was described in 2018, and additional studies characterize it as dysfunction of the retinal pigment epithelium centered on the posterior pole, which can progress despite drug cessation. Requisite exposure can be as little as 0.325 kg and 2.25 years but averages closer to 1–2 kg and 10–15 years. Multimodal imaging should include near-infrared reflectance, optical coherence tomography, and fundus autofluorescence. Cross-sectional studies demonstrate evidence correlating cumulative dosing and the likelihood/severity of maculopathy. Early estimates of prevalence range from 12.7 to 41.7% depending on dosing, with overall rates around 20%. Summary Reasonable evidence associates maculopathy with extended exposure to PPS, with an average reported incidence of around 20% in patients with long-term exposures. Patients with unexplained retinal pigment epithelium changes and difficulty with dark adaptation should be questioned regarding PPS exposure, and patients with known exposure to PPS should be examined. Further research is needed to refine screening protocols. Currently, providers should consider baseline examination and examination at 5 years and/or 500 g of exposure followed by yearly screening. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.