Ophthalmologists need to be aware that long-term use of pentosan polysulfate sodium (PPS; Elmiron, Janssen Pharmaceutical) is strongly associated with a vision-threatening maculopathy that masquerades as age-related macular degeneration (AMD) and pattern dystrophy, according to Nieraj Jain, MD.

Speaking at the American Academy of Ophthalmology 2019 Retina Subspecialty Day, Dr. Jain described the investigations that led to the recent identification of this drug-related retinal toxicity and described the clinical features that support its diagnosis. Noting that PPS was FDA approved in 1996 and is widely used to treat interstitial cystitis, a condition that is estimated to affect approximately 1 million adults in the United States,

Dr. Jain encouraged his colleagues to maintain an index of suspicion for PPS maculopathy going forward, but also to look back for cases that were misdiagnosed.

“We are all worried about missing another case of drug toxicity in the future, but I would like everyone to focus on the cases we have seen in the past,” said Dr. Jain, assistant professor, Emory Eye Center, Emory University, Atlanta, GA.

Dr. Jain explained that PPS has been on the market for 20 years and that most affected patients are already out there.

“If you have the capability of searching your electronic medical record, please do search for this drug and try to identify patients with this maculopathy,” he said.

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