Clinical Ophthalmology (Dec 2021)
Pentosan Polysulfate Sodium-Associated Pigmentary Retinopathy: Risk Factors and Fundus Findings
Abstract
Ella H Leung,1 Sahana Sharma,2 Ana Levie-Sprick,1 Gregory D Lee,1 Hyung Cho,1 Krishna Mukkamala1 1Georgia Retina, Atlanta, GA, USA; 2Vanderbilt University, Nashville, TN, USACorrespondence: Ella H LeungGeorgia Retina, 833 Campbell Hill St NW, Suite 300, Marietta, GA, 30060, USATel +1-770-218-1888Email [email protected]: To evaluate the risk factors and fundus findings of patients with potential PPS-associated retinopathy.Patients and Methods: A retrospective chart review was performed of patients exposed to PPS who had a dilated fundus examination at a large retina-only practice from 2018– 21. Multimodal images were evaluated by masked reviewers.Results: A total of 148 patients were included, of whom 33 (22%) had PPS-associated retinopathy, and 115 (78%) did not. The mean age was 60.3 years old, and the mean follow-up was 11.8 months. The PPS-associated retinopathy group had higher mean cumulative doses of PPS (1600g± 849 vs 864g± 852, P < 0.0001, Mann–Whitney test) and longer duration of PPS use (13.6 years vs 7.48, P < 0.0001). There was no statistically significant difference based on a history of kidney or liver disease or the dosage per day for the weight, body mass index, body surface area, or lean body weight. Of the patients with PPS-associated retinopathy whose genetic results were available, 15 of 16 (93%) were heterozygous for variants of uncertain significance.Conclusion: A longer duration of PPS use and higher cumulative dosage of PPS were associated with an increased risk of developing PPS-associated pigmentary retinopathy. The role of genetic mutations in patients exposed to PPS is still to be determined.Keywords: pentosan polysulfate sodium, pigmentary retinopathy, pharmacotoxicity, inherited retinal dystrophies, genetics
