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May 06, 2021
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Check for maculopathy in patients being treated for interstitial cystitis

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ATLANTA – Chronic exposure to a drug used to treat interstitial cystitis has recently been linked to pigmentary maculopathy, Nate Lighthizer, OD, FAAO, said here at SECO during a talk on the ocular effects of systemic medications.

“Interstitial cystitis is a chronic regional pain syndrome of the bladder and pelvis that predominantly affects females, manifesting with urinary urgency and dysuria (painful urination),” Lighthizer said. “Elmiron (pentosan polysulfate, Janssen) is the only oral treatment available for this condition.”

Researchers at Emory University looked at 219 patients with interstitial cystitis, “and everyone who had pigmentary maculopathy was taking pentosan polysulfate (PPS),” he said.

In the study, the mean age of ocular toxicity was 60 years, and the median duration of PPS intake was 15 to 18 years, Lighthizer said.

Nate Lighthizer, OD, FAAO
Nate Lighthizer

“The study also suggested that chronicity of exposure plays a role,” he continued. “At the 5-year time point of continuous treatment, there was a trend for increased risk, although it wasn’t statistically significant.”

The most common presenting symptoms were blurred vision (48%), prolonged dark adaptation (48%) and metamorphopsia (11%). Median duration of symptoms was 4 years, and median visual acuity was 20/25.

Clinical findings include a combination of hypo- and hyperfluorescence and retinal pigment epithelial thickening as seen on OCT, he said.

According to guidelines developed by Emory University, a baseline exam for these patients should consist of dilated fundus exam, OCT, fundus photos and fundus autofluorescence (FAF), “the most important test,” Lighthizer said. An annual exam should be conducted after 5 years of medication use.

Watch patients closer if there is concurrent macular disease, renal or hepatic issues, or unusually high dosages of medication, he said. If toxicity is seen, discuss discontinuing the treatment with the prescriber and the patient.

Lighthizer added that many of these cases of Elmiron toxicity may have previously masqueraded as other similar-looking conditions such as age-related macular degeneration and pattern dystrophy.

“The fundus findings in PPS-associated maculopathy are subtle yet exhibit a distinctive clinical phenotype on multimodal imaging that’s best appreciated by using FAF,” he concluded.