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.
Highlights
Pentosan polysulfate sodium (PPS) is an oral drug used for interstitial cystitis (IC), a chronic urinary pain syndrome thought to be due to irritation of the bladder interstitium
Among these 216 patients, 96 patients had a documented ophthalmic examination at the University of California Davis Eye Center (Table 1). Of these 96 patients, 24 had macular imaging which was reviewed for the retinal abnormalities noted in the original report [2]: (1) autofluorescent imaging revealing a densely packed array of hyperautofluorescent and hypoautofluorescent spots involving the posterior pole, (2) fundus photography revealing macular hyperpigmented spots, yellow-orange deposits, and/or patchy retinal pigment epithelium (RPE) atrophy, and (3) optical coherence tomography (OCT) imaging demonstrating focal thickening or elevation of the RPE with associated hyperreflectance on near-infrared reflectance imaging
One mechanism that we hypothesize is that circulating PPS may disrupt or deposit in the underlying Bruch’s membrane of the retinal pigmented epithelium (RPE) and photoreceptor cells (PC)
Summary
Pentosan polysulfate sodium (PPS) is an oral drug used for interstitial cystitis (IC), a chronic urinary pain syndrome thought to be due to irritation of the bladder interstitium. In 2018, a case series of 6 patients demonstrated a possible link between long-term PPS exposure and pigmentary maculopathy [2]. In 2019, a multi-institutional case series identified 35 patients across 4 institutions with long-term exposure to PPS and pigmentary maculopathy [3]. An additional case report of maculopathy after long-term exposure to PPS was found to continue to progress for 6 years after discontinuation of this medication resulting in severe vision loss [4]. In an additional case series, 11 patients with long-term PPS exposure and development of pigmentary maculopathy were followed after cessation of PPS for at least six months. No eyes had a marked improvement in disease following cessation and 9 of these 11 patients reported worsening visual symptoms at the final follow-up [5]
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