Association between a newly described pigmentary maculopathy and pentosan polysulfate sodium

Abstract

Purpose : Interstitial cystitis is a chronic regional pain syndrome of the bladder affecting millions of individuals in the United States. Presently, only two FDA-approved therapies exist: oral pentosan polysulfate sodium (PPS) and intravesical dimethyl sulfoxide. Recently, we described a unique pigmentary maculopathy in a group of patients undergoing management of interstitial cystitis with PPS. In this study we identified PPS exposure amongst patients with interstitial cystitis and hypothesized that this novel maculopathy would manifest with greater odds in those cases with PPS exposure.

Methods : A retrospective cross-sectional study of patients with a diagnosis of interstitial cystitis seen at the Emory Eye Center between May 2014 and October 2018 was performed. Records were evaluated for use of PPS and features of the suspected maculopathy on ophthalmic imaging. Posterior segment images were assessed by masked graders for resemblance to the characteristic maculopathy according to a predefined case definition and classified as follows: 1 (Affected), 2 (Possibly Affected), 3 (Unaffected), 4 (Insufficient Imaging).

Results : 216 patients with interstitial cystitis were identified (Table 1). Of these, 89 had documented exposure to pentosan polysulfate sodium. In this group, 14 (15.7%), 7 (7.9%), 25 (28.1%), and 43 (48.3%) patients received Grades 1, 2, 3, and 4, respectively. In the unexposed group, 0, 4 (3.1%), 23 (18.1%), and 100 (78.7%) patients received Grades 1, 2, 3, and 4, respectively. Because 0 patients in the unexposed group were designated Grade 1, no odds ratio associating Grade 1 scores could be computed. Grade 1 and 2 patients were thus consolidated into one “unspecified pigmentary maculopathy” group. The odds ratio of developing an unspecified pigmentary maculopathy with PPS exposure was 9.50 (95% confidence interval 3.13 – 28.80; P = 0.0001).

Conclusions : Exposure to PPS is strongly associated with development of a pigmentary maculopathy in patients with interstitial cystitis. In fact, patients designated “Grade 1” for the unique PPS-associated maculopathy were identified exclusively in the PPS-exposed group. We recommend drug cessation in affected patients.

This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.