Late Development of Clinically Apparent Pentosan Polysulfate Maculopathy after Drug Cessation

To describe two cases of delayed onset pentosan polysulfate (PPS) maculopathy that became clinically apparent years after drug cessation.

Two patients followed with serial ophthalmic examination and multimodal fundus imaging.

Two women, each with > 2400 g cumulative exposure to PPS, developed clinically apparent PPS maculopathy years after drug cessation. In each case, baseline fundus imaging soon after PPS cessation demonstrated a thin choroid and subtle nonspecific fundus irregularities, but not the classic features of toxicity.

These cases suggest that late development of clinically detectable pentosan polysulfate maculopathy may occur years after drug cessation. These findings corroborate earlier work suggesting that choroidal changes may be an early but nonspecific sign of toxicity. This observation has important ramifications for PPS dosing and surveillance guidelines.