This 64YOF presented in February 2020 with findings of Pentosan polysulfate sodium (PPS, Elmiron) retinal toxicity. She used PPS for 4 years, but discontinued the drug 9 years earlier. We have since followed her yearly.
Optos color imaging of the prior (17 months earlier) and current examinations shows variable yellow-orange atrophic macular pigmentary changes. These findings are best visualized with fundus autofluorescence.
Close inspection of these images shows definite enlargement of the RPE atrophy, especially in her left eye. She remains completely free of visual symptoms with vision of 20/30 OD and 20/25 OS.
Learning Points:
Elmiron was approved by the FDA in 1996 for treating interstitial cystitis. A unique PPS retinopathy was recently described, and our patient shows classic findings.
Toxicity seems to develop over many years and can mimic more common disorders, including age-related macular degeneration and macular dystrophies.
Progressive changes, as in our patient, usually continue despite discontinuation of the causative drug (Shah et al., JAMA Ophthalmology 2020;138:894-900).
