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Joseph Christiansen, Alexander Barnes, Duncan Berry, Nieraj Jain; Pentosan polysulfate maculopathy versus age-related macular degeneration: comparative assessment with multimodal imaging. Invest. Ophthalmol. Vis. Sci. 2020;61(7):1062.
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Due to similarities in demographic characteristics and fundus appearance of these conditions, many patients with pentosan polysulfate maculopathy are initially diagnosed with age-related macular degeneration. This study investigates the hypothesis that pentosan polysulfate maculopathy manifests distinctive imaging features that can be differentiated from those found in age-related macular degeneration (AMD).
Several local databases were queried to identify patients seen at the Emory Eye Center between May 2014 and January 2019 with a diagnosis of interstitial cystitis. Records of the identified patients were reviewed to determine those that had adequate imaging that would allow for further classification. Ninety subjects met these eligibility criteria. Masked graders categorized patients based on imaging characteristics as follows: Category 1 – pentosan polysulfate maculopathy; Category 2 – AMD or drusen; Category 3 – neither; Category 4 – unsure.
Of the ninety subjects evaluated, 79 (88%) were female and the median age was 61.5 years (range, 30 - 89). There were 17 subjects placed in category 1 (PPS maculopathy), and all 17 (100%) had exposure to PPS. There were 25 patients in category 2 (AMD or drusen) and 15 (60%) had PPS exposure, 47 patients in category 3 (neither) and 28 (60%) had exposure to PPS, and 1 patient in category 4 (unsure) and this patient had no PPS exposure. Mean cumulative exposure to pentosan polysulfate across categories 1-4 was 2.1kg, 0.36kg, 0.34kg, and 0 kg respectively (p<0.00001). Subjects with pentosan polysulfate maculopathy did not have typical drusen in the macula.
Although there are some similarities between pentosan polysulfate maculopathy and age-related macular degeneration, these two entities can be differentiated with the use of multimodal fundus imaging.
This is a 2020 ARVO Annual Meeting abstract.
Representative case of PPS maculopathy in a 63 year old female with 2.48 kg cumulative exposure to PPS. This patient has characteristic macular pigment clumps (A) amidst a background of pale yellow fundus deposits. Near infrared reflectance imaging (B) and fundus autofluorescence imaging (C) demonstrate the extent of involved tissue, with a fairly dense arrangement of signal abnormalities in the macula. Macular pigment clumps are associated with corresponding hyperreflective nodules at the level of the retinal pigment epithelium (D).
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