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. 2021 Feb 11:15:513-519.
doi: 10.2147/OPTH.S285013. eCollection 2021.

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

Neil S Kalbag  1 , Nenita Maganti  1 , Alice T Lyon  1 , Rukhsana G Mirza  1
Affiliations

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

Neil S Kalbag et al. Clin Ophthalmol. .

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.

Keywords: IC; PPS; interstitial cystitis; maculopathy; pentosan polysulfate sodium.

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Conflict of interest statement

The authors have no proprietary or commercial interest in any of the materials discussed in this article and report no conflicts of interest in this work.

Figures

Figure 1
Figure 1
(Patient 9) Fundus photography: Fundus autofluorescence at early stage of disease showing pigment mottling, reticular hypoautofluorescent and hyperautofluorescent spots, and focal areas of RPE enlargement.
Figure 2
Figure 2
(Patient 8) OCT: Later stage of the disease shows large areas of confluent RPE atrophy.
Figure 3
Figure 3
(Patient 8) Fundus autofluorescence at presentation and 4 years later, depicting the increase in areas of RPE atrophy.
See this image and copyright information in PMC

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