Progression of pentosan polysulfate sodium maculopathy following discontinuation

Examination of a 69-year-old woman with a family history of age-related macular degeneration (AMD) revealed bilateral macular atrophy with surrounding deposits evident on fundus autofluorescence (FAF). The extent of macular atrophy in this 69-year-old, lack of typical drusen, and FAF pattern did not support a diagnosis of AMD. Her clinical presentation was consistent with pentosan polysulfate sodium (PPS) maculopathy. Further questioning revealed that she had been taking 400 mg of PPS daily for 7 years (cumulative dose ~1000g) but had stopped 2 years ago. Review of available previous images demonstrated continued progression of the maculopathy after discontinuation of PPS. FAF demonstrated atrophy in both eyes 18 months after PPS discontinuation, as well as its progression over the subsequent 6 months. Her clinical presentation was consistent with PPS maculopathy, with evidence of progressive atrophy despite PPS discontinuation 2 years earlier.