This 65YO female was taking Elmiron (pentosan polysulphate, PPS) for many years beginning in the mid-1990s. Vision was 20/400 OD and counting fingers OS.
Variable yellow-orange atrophic pigmentary changes were noted funduscopically throughout each macula, with more dramatic patches of macular hypoautofluorescence with a peripheral granular hyper- and hypoautofluorescent appearance.
OCT scanning showed variable outer retinal loss.
Learning Points:
Elmiron was approved by the FDA in 1996 for treating interstitial cystitis. Recently, a unique PPS retinopathy has been 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.
Peripapillary hypoautofluorescence, more densely packed macular autofluorescent changes, and earlier central macular involvement suggest PPS toxicity over other causes (see Barnes et al, Ophthalmology Retina 2020;4:1196-1201).
