Abstract
Purpose :
Recent studies describe a novel, dose-dependent association between pentosan polysulfate sodium/PPS, an oral glycosaminoglycan used to treat interstitial cystitis (IC), and pigmentary maculopathy. Although existing literature suggests a progressive, vision-threatening macular condition associated with long-term PPS use, no observational data have included a group of patients with IC who have not been exposed to PPS. This study examines whether this is a drug-associated toxicity or a manifestation of the disease itself.
Methods :
A retrospective review was performed of all patients with IC that have undergone at least two eye examinations at a large academic center over the last ten years. Patients were excluded from analysis if they had other visually significant pathology besides cataracts, AMD or other maculopathies, if they did not have a dilated fundus exam, or if they had a history of amblyopia. A blinded grader reviewed the retinal imaging (any combination of OCT, FAF, or fundus photography) of all patients with imaging data and assessed for presence of PPS maculopathy.
Results :
Of the 337 patients with IC included in the study, 178 (53%) had PPS exposure. Median duration of PPS intake was 29 (range: 0.1-134) months. The median daily PPS dosage was 300 (range: 100-400) mg, and the mean cumulative PPS exposure was 411 (381 SD, range: 0.8-1481) g in the ten-year study period. The mean initial and final visual acuity did not differ significantly between the exposure and non-exposure groups. Of the 151 patients (302 eyes; 12 eyes with exposure, 290 without) with imaging data, 6 patients (12 eyes) were identified to have signs of PPS maculopathy, all of whom were part of the exposure group. 3 patients (6 eyes) were identified to have mild maculopathy (cumulative doses 34g, 117.2g, 1349.6g), one patient (both eyes) had moderate maculopathy (929.2g), one patient (both eyes) had severe maculopathy (1109.1g) while one patient had one eye affected with moderate maculopathy and the other with severe maculopathy (475.6g).
Conclusions :
Our data show that the characteristic macular changes previously associated with PPS use were only observed in IC patients with PPS exposure and not in patients without PPS exposure. While visual acuity is relatively well-preserved in patients with PPS exposure, there was a trend of progressive, dose-dependent drug-associated maculopathy in the six patients affected.
This abstract was presented at the 2023 ARVO Annual Meeting, held in New Orleans, LA, April 23-27, 2023.