June 5, 2020 Conference


Features of pentosan polysulfate sodium-associated maculopathy in a diverse patient population

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Gabrielle Fridman (Presenter)
Dr. Ka Yi Emily Tam, BMC
Dr. Alexander Port, Boston Medical Center
Dr. Nicole Siegel, -
Purpose: To characterize clinical features of presumed pentosan polysulfate sodium (PPS)-associated maculopathy in a diverse patient population. Methods: Retrospective chart review of 254 charts. The electronic medical record (EMR) was queried to identify all patients with a history of pentosan polysulfate sodium exposure and a documented dilated eye examination between 2011-2019. Patient demographics, ocular findings, PPS exposure and dosage were reviewed. Subjects were included in analysis if PPS exposure, eye examination and optical coherence tomography (OCT) imaging were documented in the EMR. Results: Seven patients were identified who had both exposure to PPS and a pigmentary maculopathy on the fundus exam (Table 1). Similar to prior reports, our patients described symptoms of blurry vision at either distance or near despite having excellent Snellen best corrected visual acuity (BCVA). Age of presentation was comparable to prior reports with a mean of 53 years and a range from 43 to 73 years. All patients showed macular retinal pigment epithelium (RPE) mottling or atrophy on the clinical exam and OCT. Unique to our series we identified a larger proportion of non-Caucasian females and the second male, to the authors knowledge, to be affected by PPS. Conclusions: PPS-associated maculopathy is a recently described condition with poorly characterized risk factors and natural history. PPS-maculopathy appears to affect both men and women. Clinicians should inquire about PPS, which is most commonly prescribed for interstitial cystitis, in patients with macular changes or RPE atrophy of unclear cause or atypical presentation. Increased awareness of this condition will help to ensure proper screening, diagnosis and treatment. Further investigation is warranted to elucidate the pathophysiology as well as establish screening and treatment guidelines.
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