RAPID PROGRESSION OF MACULOPATHY AFTER PENTOSAN POLYSULFATE SODIUM CESSATION

To report a case of pentosan polysulfate sodium (PPS) maculopathy showing rapid progression of retinopathy 4 years after discontinuing PPS.

A 75-year-old woman presenting with gradual vision changes underwent comprehensive examination including multimodal imaging and genetic testing.

A 75-year-old woman presented with a history of intermediate age-related macular degeneration and progressive blurring of her central vision beginning at 69 years of age. Fundoscopic examination revealed retinal pigment epithelium abnormalities and multifocal outer retinal loss. The patient had been first prescribed PPS for interstitial cystitis at 63 years of age with a cumulative PPS dose of approximately 1,300 g and ceased PPS at 71 years of age. She was noted to have only minimal retinal changes before drug cessation. Four years postcessation, the patient reported marked night vision and color vision abnormalities and was noted to have severe retinal changes including macular atrophy. Genetic testing revealed the patient to be homozygous for the previously reported risk alleles.

PPS maculopathy is an acquired toxic disease that can significantly impact a patient's quality of life. Our report highlights that marked progression of PPS retinopathy can occur even after drug cessation and that patients need to be carefully monitored and given supportive management.