Pentosan polysulfate sodium maculopathy: Final analysis of a prospective cohort, updated review, and association with inflammatory bowel disease.

Pentosan polysulfate sodium (PPS) maculopathy is a recently described condition of macular toxicity. We report the prevalence (15%), the clinical characteristics, and the multimodal imaging (MMI) features of PPS maculopathy based on a final prospective analysis of a cohort of 154 subjects and highlight the association with inflammatory bowel disease (IBD). In addition, we summarize the most recent and relevant literature on PPS maculopathy. This study and other recent analyses provide solid evidence of the toxic effects of PPS on the retina and choroid and refute the concept that the maculopathy is an association of the underlying disease, interstitial cystitis. Moreover, this study and others raise a major concern that PPS may also be toxic to the bowel. Vigilant surveillance of patients receiving this potentially harmful drug is critical. We review the recommended screening guidelines, the pathophysiology and MMI spectrum of disease, the differential diagnosis including MMI clues to an accurate diagnosis, and the progression of retinopathy that can take place despite discontinuation of the drug, emphasizing the critical importance of early detection of toxicity.