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Malini Veerappan Pasricha, Cassie A. Ludwig, Jose Davila, Darius Moshfeghi; The association between pentosan polysulfate sodium exposure and drug-induced maculopathy. Invest. Ophthalmol. Vis. Sci. 2020;61(7):1065.
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Several studies have demonstrated an association between pentosan polysulfate sodium (PPS, a drug used to treat interstitial cystitis) and a unique pigmentary maculopathy. In this study, we present a detailed evaluation of limited cases, all with chronic exposure to PPS. The purpose is to describe the key clinical features of PPS-associated maculopathy.
This is a retrospective cohort study. All patients with a history of PPS use and an ICD-9 or ICD-10 diagnosis of exudative or nonexudative age-related macular degeneration, toxic maculopathy, hereditary maculopathy, or drusen were identified using the Stanford University Medical Center clinical data warehouse. The primary outcome was key retinal features based on multimodal imaging. A secondary outcome was the clinical spectrum of disease based on daily dose by weight and duration of exposure.
Thirteen patients (n=26 eyes) were identified. Average age was 65.1 years, 10/13 (76.9%) were females and 8/13 (61.5%) were white. Daily PPS dose by weight ranged from 2.9 to 6.2 (mean 4.4) mg/kg, and average duration of exposure ranged from 60 to 4745 (mean 919.4) days. Most patients (10/13, 76.9%) presented to the eye clinic with a chief complaint of progressive blurry vision. On funduscopic exam and photo, key pathology noted were mild retinal pigment epithelium (RPE) mottling (n= 3 eyes), pigment changes (n= 6 eyes), and central, peripapillary and/or peripheral drusen (n=16 eyes). On optical coherence tomography of the macula (OCTmac), features present in two or more eyes were hypo- and hyper-pigmented perifoveal spots at the RPE level, subfoveal drusen, subretinal fluid, RPE atrophy, and choroidal neovascular membrane. Patients with a higher daily dose by weight did not appear to have more advanced pathology or poorer visual acuity. Patients who had a longer duration of drug exposure had more pathology on funduscopic findings, but not poorer visual acuity.
Key clinical features of PPS-associated maculopathy are RPE mottling and pigment changes perifoveally, and drusen in central or peripheral location. On OCTmac, most common features are hypo and hyperpigmented perifoveal spots at the level of the RPE, subfoveal drusen, subretinal fluid, and RPE atrophy.
This is a 2020 ARVO Annual Meeting abstract.
Representative imaging from Patient SL. Top row: color fundus photo, left and right eye. Bottom row: optical coherence tomography of the macula, left and right eye.
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