This 74YO female had a long history of Elmiron (pentosan polysulfate, PPS) use. Vision was 20/30 OU.
Variable yellow-orange atrophic pigmentary changes were noted throughout each macula, with more dramatic patches of macular and peripapillary hyper- and hypoautofluorescence.
Triton swept-source OCT showed some patchy ellipsoid loss OD and diffuse hyperreflectivity of the outer segments.
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), including MIDD. Mitochondrial disease, pattern macular dystrophies, and PPS maculopathy have similar yet distinct findings. We hope the last 3 days of posts will help to clarify these clinically overlapping phenotypes.
