Identification of Patients with Pentosan Polysulfate Sodium-Associated Maculopathy through Screening of the Electronic Medical Record at an Academic Center

Abstract

Aims. This chart review of a quaternary academic medical center electronic medical record (EMR) aimed to identify patients at risk of development of maculopathy with exposure to pentosan polysulfate sodium (PPS). Methods. A review of electronic medical records of a quaternary medical center of patients with either documented exposure to PPS or diagnosis of interstitial cystitis (IC) from 2007 to 2019 was performed for retinal imaging and visual acuity; the study was conducted in August of 2019. Results. 216 charts were included for analysis, of which 96 had documented eye exams and 24 had retinal imaging done. We identified three patients with maculopathy in the context of long-term exposure to PPS via chart review, and one additional patient was identified by referral. The median PPS exposure duration was 11 years (range 7 to 19 years). Median logMAR BCVA OD 0.6 range was 0.0–1.9 (approximate Snellen equivalent 20/80 range (20/20–20/1600)) and OS 0.7 range was 0.1–1.9 (approximate Snellen equivalent 20/100 range (20/25–20/1600)). Ultrawidefield color fundus imaging and fundus autofluorescence revealed findings of pigmentary changes and patchy macular atrophy. Optical coherence tomography (OCT) demonstrated outer retinal thinning and increased choroidal transmission coincident with areas of atrophy seen on fundus imaging. Conclusions. Less than half of patients at risk for development of maculopathy due to exposure to PPS had received eye examinations, suggesting that those at risk are not receiving adequate screening. We found two patients with PPS maculopathy who had relatively preserved central vision, one patient with bitemporal vision loss, and one patient who developed vision loss in both eyes.

Pigmentary Maculopathy in Interstitial Cystitis/Bladder Pain Syndrome Treated with Oral Pentosan Polysulfate: A Review

“…The percentage of patients who received PPS and were later diagnosed with maculopathy is estimated to be 2.4%. A similar prevalence of 2.0% (4 out of 216) was reported by Higgins et al, 49 who conducted a chart review of a quaternary academic medical centre electronic medical record database. Kalbag et al 50 described a prevalence of 1.5% (10 out of 131) based on electronic health record data.…”

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

“…These patients also had significantly greater mean duration of exposure, daily dose, and cumulative dose. Finally, Higgins et al 26 showed a significant dose-response relationship between PPS exposure and presence of maculopathy in 3 out of 96 PPS users at the University of California at Davis.…”

“…These nodules may not be present in advanced disease when RPE atrophy occurs 1,25,33 . Some cases also show intraretinal hyperreflective foci like those seen in AMD that may represent dissociated RPE cells 26 . Many cases show outer retinal irregularity, retinal thinning, and RPE atrophy 25,33 …”

“…1,25,33 Some cases also show intraretinal hyperreflective foci like those seen in AMD that may represent dissociated RPE cells. 26 Many cases show outer retinal irregularity, retinal thinning, and RPE atrophy. 25,33 Investigators have also noted the possible importance of near-infrared reflectance (NIR) in screening for PPS maculopathy.…”