Maculopathy Secondary to Pentosan Polysulfate Use: A Single-Center Experience

Abstract

Aim: To investigate the prevalence of retinal pathology in patients with a history of exposure to pentosan polysulfate sodium (PPS). Methods: Patients exposed to PPS and seen in the ophthalmology clinic at Northwestern University during 1/1/2002 to 1/1/2019 were identified from electronic health records (EHR) by an electronic data warehouse (EDW) search. Visual acuity (VA), reasons for clinic visit, ocular conditions, and duration of exposure to PPS were noted. Chart review was performed for fundus exam findings and ophthalmologic imaging, specifically fundus photography, fundus autofluorescence, and ocular coherence tomography (OCT) images. When OCT or fundus photography was available, studies were evaluated by two independent graders. Results: A total of 131 patients who were exposed to PPS and seen at the Northwestern Ophthalmology clinic were identified in the EHR. Forty patients of 131 had imaging. Patients with imaging or fundus examination suspicious for PPS maculopathy were placed into the suspect group. Of the 40 patients that had imaging, 5 (12.5%) had features suspicious for PPS maculopathy. Of the remaining 91, 5 (5.4%) had macular pigmentary changes described on fundus exam. Among the 10 patients in the suspect group, the average duration of PPS use was 4.2 years (range 0.3-11.6 years, interquartile range 5.5 years) and the average cumulative dose was 380g (range 29-1092g, interquartile range 132g). Conclusion: A novel drug-induced maculopathy has been associated with PPS use with a distinct clinical constellation that can be accurately identified with multimodal imaging.

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

“…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.…”

Pentosan polysulfate maculopathy: Keep an eye out for this masquerader

“…7 Subsequent studies independently conducted at numerous centers have reported a significant dose-response relationship between PPS exposure and presence of maculopathy. [8][9][10][11][12][13] A study from 2020 demonstrated prevalence rates of 13%, 30%, and 42% among patients with 500-999 grams (g), 1000-1499 g, and >1500 g of cumulative PPS exposure, respectively. 8 In another study from earlier this year, researchers found prevalence rates of 46% and 83% among patients with cumulative PPS exposures of 1500-2000 g and ≥2000 g. 11 Studies of large administrative claims databases have yielded mixed findings.…”