This 74YO female has been followed by us for a few years with known pentosan polysulfate (PPS, Elmiron) retinal toxicity. She has been taking Elmiron for 25 years.
When we initially diagnosed her with these retinal findings, she was hesitant to stop treatment since her interstitial cystitis symptoms were intolerable and dramatically improved with this medicine. She returned for her 6-month examination on 1/27/23 with increased central visual and contrast complaints.
Optos color imaging looked fairly stable. However, fundus autofluorescence (FAF) shows definite progressive macular changes. Macular color photography with swept-source OCT shows variable loss of the outer retinal layers with some focal outer retinal hyperreflective deposits.
Learning Points:
Elmiron was approved by the FDA in 1996 for treating interstitial cystitis. A unique PPS retinopathy was described in 2018, 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.
The drug should be discontinued once retinal findings are discovered, although progressive functional and structural changes can develop even after drug cessation (Jung et al, JAMA Ophthalmol 2023;141:260-266).
We strongly recommend that she discontinue her Elmiron therapy. However, she felt that she would rather completely lose her central vision than face life with the pain from untreated interstitial cystitis and will remain on treatment.
She will discuss other potential therapeutic options with her nephrologist in the meantime.
