Advanced pentosan polysulfate sodium maculopathy with low cumulative exposure and hydroxychloroquine use.

Purpose: To report a case of pentosan polysulfate sodium (PPS) maculopathy with cystoid macular edema (CME) with relatively low cumulative PPS exposure and a history of concurrent hydroxychloroquine use. Observations: A 46-year-old female was treated with PPS daily for 10 years, and hydroxychloroquine intermittently over a span of five years, actively taking hydroxychloroquine for a sum of three years during PPS therapy. Despite a low risk for toxicity based on the cumulative exposure to either medication, fundoscopic examination and multimodal imaging revealed severe PPS maculopathy with CME two and a half years post-PPS cessation. CME was refractory to topical therapy and intravitreal anti-VEGF but improved with intravitreal dexamethasone. Bilateral improved visibility of the ellipsoid zone (EZ) was observed on Spectral Domain Optical Coherence Tomography (SD-OCT) following dexamethasone injection. Conclusions and importance: Many reports describe an association between cumulative PPS exposure and maculopathy; however, risk factors that may contribute to PPS-associated maculopathy in the setting of low PPS exposure are not well characterized. This case indicates that other risk factors implicated in retinal pigment epithelium dysfunction should be investigated, including concurrent hydroxychloroquine use.