As a health publishing company dedicated to interstitial cystitis, it is our duty to share news which can impact you, the IC patient. Unfortunately, we must share some distressing news. If you have or are currently taking pentosan polysulfate PPS (brand name: Elmiron®) and are experiencing eye issues, researchers at the Emory Eye Center (Emory School of Medicine, Atlanta, GA) have now documented cases of retinal maculopathy (aka retinal injury) in SOME IC patients who have used pentosan for long periods of time. They urge patients who have used or are using pentosan to be screened by ophthalmologists if they are experiencing any unusual eye symptoms (difficulty reading, vision loss, difficulty seeing in lower lights) and to cease pentosan therapy if eye disease is present.
2018 – First Case Series & Report
In Spring 2018, Dr. Neiraj Jain and collages wrote a letter to the editor of the Journal of Urology that stated “We wish to alert readers to a concerning new observation of vision threatening retinal changes associated with long-term exposure to PPS. We recently reported our findings of retinal pigmentary changes in six patients undergoing long-term therapy with PPS….Each patient had received a standard dosage of PPS, ranging from 200 to 400 mgs daily, for a median duration of 15.5 years (range 12 to 20) … Examination findings of patients with this condition are suggestive of injury to the retina and the underlying retinal pigment epithelium. After extensive investigations, which included molecular testing for hereditary retinal disease, we found these cases to resemble no other retinal disease.”(1)
IC experts Robert Moldwin and Curtis Nickel responded with concern, stating “It is quite unlikely that urologists treating patients with IC/BPS ever would have made this association…. yet the implications are either frightening if our treatment is causing this condition or instructive if this condition is a previously unknown manifestation of IC/BPS.”(2) They noted that the Emory Eye Center had identified additional cases in their clinic as well as in the clinics of colleagues.
“Pigmentary Maculopathy Associated With Chronic Exposure to Pentosan Polysulfate Sodium” was published in the Journal of Ophthalmology in May 2018. A case series, it focused on six women with interstitial cystitis who presented to their clinic between May 2015 and October 2017 with pigmentary maculopathy.(3) A maculopathy is condition which affects the center of the retina, an area associated highly sensitive and accurate vision, such as Age-Related Macular Degeneration.(4) Most of these patients had difficulty reading and difficulty in darkness. Two patients experienced a generalized dimming of their vision as the first symptom. Two others had difficulty with near vision. One had paracentral scotomas (vision loss) in part of their eye while the other had metamorphopsia (distorted vision where straight lines become wavy).
All six patients underwent rigorous diagnostic imaging and DNA testing to determine if they had any genes associated with hereditary retinal loss. None had a family history of retinal disease nor the discovery of any pathogenic process. What they did have in common was the use of pentosan (aka Elmiron®) for long periods of time (a median of 186 months.) Five had been treated at a dose of 400mg daily (two of whom reduced their dosage to 200mg daily after 17 years). One patient was treated with a 300mg daily dose. No other medications were used by more than 2 patients in the study.
Examinations of their eyes showed clear changes “Nearly all eyes (10 eyes of 5 patients) showed subtle parafoveal pigmented deposits at the level of the retinal pigment epithelium (RPE).” All eyes “showed subtle viteliform deposits that increased in number and extended beyond the major arcade of vessels in cases judged to be more severe. Four eyes of 2 patients showed RPE atrophy that was noted to increase in area and encroach on the central fovea over time. “ Findings were “fairly symmetrical” between the eyes of each patient. Retinal imaging also found clear diseased regions and/or atrophy though the size varied among patients.
It’s important to note that youngest patient in the study was just 37 years old. Patient #4 diagnosed at the age of 23. She was taking 400mgs of Pentosan a day. She began showi