Abstract
Pentosan polysulfate sodium (PPS), a medication traditionally prescribed for interstitial cystitis, has more recently been used for alternative indications. Chronic exposure and high cumulative dosing are now recognized as risk factors for the development of a distinctive and irreversible maculopathy. We report the case of a patient who developed bilateral vision loss initially attributed to non-exudative age-related macular degeneration but was later found to have characteristic features of PPS maculopathy after a detailed history revealed seven years of off-label subcutaneous PPS injections for osteoarthritis-related back pain. Multimodal imaging, including spectral-domain OCT and fundus autofluorescence, demonstrated hallmark findings such as hyperpigmented macular spots, RPE atrophy, hyperreflective nodules, and a peripapillary hypoautofluorescent halo. This may represent the first reported case of PPS maculopathy resulting from subcutaneous administration. This case underscores the diagnostic challenge of PPS toxicity, its potential to mimic age-related macular degeneration, and the importance of heightened awareness of non-oral administration routes and off-label PPS use. Early recognition and ophthalmologic screening are essential to prevent irreversible vision loss in patients exposed to PPS.
Presentation Date: 09/25/2025
Issue Date: 02/27/2026
