Phenotypic Spectrum of Pentosan Polysulfate Sodium–Associated Maculopathy

Hanif, Adam M.; Armenti, Stephen T.; Taylor, Stanford; Shah, Rekha D.; Igelman, Austin D.; Jayasundera, Thiran; Pennesi, Mark E.; Khurana, Rahul N.; Foote, Jenelle; O’Keefe, Ghazala; Yang, Paul; Hubbard, G. Baker; Hwang, Thomas S.; Flaxel, Christina J.; Stein, Joshua D.; Yan, Jiong; Jain, Nieraj

doi:10.1001/jamaophthalmol.2019.3392

Cited by 93 publications

(151 citation statements)

References 13 publications

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“…Five had fundus autofluorescence imaging done, of which 3 had retinal imaging findings consistent with prior reports of maculopathy in the context of long-term PPS exposure [ 2 , 3 ]. Outside of the chart review, one additional patient was referred (patient 4) and found to have findings consistent with PPS-associated maculopathy ( Table 2 ).…”

Section: Resultssupporting

confidence: 59%

“…In 2018, a case series of 6 patients demonstrated a possible link between long-term PPS exposure and pigmentary maculopathy [ 2 ]. In 2019, a multi-institutional case series identified 35 patients across 4 institutions with long-term exposure to PPS and pigmentary maculopathy [ 3 ]. An additional case report of maculopathy after long-term exposure to PPS was found to continue to progress for 6 years after discontinuation of this medication resulting in severe vision loss [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

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Identification of Patients with Pentosan Polysulfate Sodium-Associated Maculopathy through Screening of the Electronic Medical Record at an Academic Center

Higgins

Welch

Bacorn

et al. 2020

Journal of Ophthalmology

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Aims. This chart review of a quaternary academic medical center electronic medical record (EMR) aimed to identify patients at risk of development of maculopathy with exposure to pentosan polysulfate sodium (PPS). Methods. A review of electronic medical records of a quaternary medical center of patients with either documented exposure to PPS or diagnosis of interstitial cystitis (IC) from 2007 to 2019 was performed for retinal imaging and visual acuity; the study was conducted in August of 2019. Results. 216 charts were included for analysis, of which 96 had documented eye exams and 24 had retinal imaging done. We identified three patients with maculopathy in the context of long-term exposure to PPS via chart review, and one additional patient was identified by referral. The median PPS exposure duration was 11 years (range 7 to 19 years). Median logMAR BCVA OD 0.6 range was 0.0–1.9 (approximate Snellen equivalent 20/80 range (20/20–20/1600)) and OS 0.7 range was 0.1–1.9 (approximate Snellen equivalent 20/100 range (20/25–20/1600)). Ultrawidefield color fundus imaging and fundus autofluorescence revealed findings of pigmentary changes and patchy macular atrophy. Optical coherence tomography (OCT) demonstrated outer retinal thinning and increased choroidal transmission coincident with areas of atrophy seen on fundus imaging. Conclusions. Less than half of patients at risk for development of maculopathy due to exposure to PPS had received eye examinations, suggesting that those at risk are not receiving adequate screening. We found two patients with PPS maculopathy who had relatively preserved central vision, one patient with bitemporal vision loss, and one patient who developed vision loss in both eyes.

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Section: Resultssupporting

confidence: 59%

Section: Introductionmentioning

confidence: 99%

Identification of Patients with Pentosan Polysulfate Sodium-Associated Maculopathy through Screening of the Electronic Medical Record at an Academic Center

Higgins

Welch

Bacorn

et al. 2020

Journal of Ophthalmology

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“…3 Subsequent studies at multiple institutions have confirmed a strong association between long-term PPS use and the newly described maculopathy while also expanding our understanding of the phenotypic manifestations of this condition. [4][5][6] Fundus examination typically reveals nonspecific findings of macular pigment clumps amidst a background of yellowish subretinal deposits in early disease and retinal pigment epithelium (RPE) atrophy in late disease. 6 Multimodal fundus imaging demonstrates characteristic findings that localize disease to the RPE and RPE-photoreceptor interface and help differentiate this condition from other maculopathies such as age-related macular degeneration (AMD) and pattern dystrophy.…”

mentioning

confidence: 99%

“…6 Multimodal fundus imaging demonstrates characteristic findings that localize disease to the RPE and RPE-photoreceptor interface and help differentiate this condition from other maculopathies such as age-related macular degeneration (AMD) and pattern dystrophy. [6][7][8] In particular, fundus autofluorescence (AF) imaging reveals a characteristic pattern of densely packed hypo-and hyperautofluorescent spots that is typically centered on and involves the fovea and is highly symmetric between eyes. 9 Visual acuity in PPS maculopathy is typically normal until late disease in the setting of RPE atrophy.…”

mentioning

confidence: 99%

Visual Function in Pentosan Polysulfate Sodium Maculopathy

Lyons

Brower

Jain

2020

Invest. Ophthalmol. Vis. Sci.

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Individuals with pentosan polysulfate sodium (PPS) maculopathy commonly report symptoms of prolonged dark adaptation and difficulty reading. We hypothesize that PPS maculopathy causes degradation of visual function not fully captured with visual acuity testing. METHODS. Subjects with PPS maculopathy underwent multimodal evaluation of retinal structure and function. Structural changes were graded as moderate or advanced. Patientreported visual function was assessed with the National Eye Institute Visual Function Questionnaire 39 (NEI-VFQ-39) and Low Luminance Questionnaire (LLQ). Objective functional evaluations included Early Treatment of Diabetic Retinopathy Study (ETDRS) bestcorrected visual acuity (BCVA), Pelli-Robson contrast sensitivity, mesopic microperimetry, and dark adaptometry. Functional testing results were correlated with structural disease category. RESULTS. Thirteen patients (26 eyes), median age 62 years (range, 37-76), completed the study. Median ETDRS letter score was 82 (Snellen equivalent 20/25). Median NEI-VFQ-39 and LLQ composite scores were 65 (range, 33-88) and 41 (range, 20-92), respectively. Median contrast sensitivity was 1.65 (range, 0.15-1.95), and median mesopic microperimetry average thresholds and percent reduced thresholds were 26 decibels (range, 0.4-28.6) and 21.6% (range, 0-100%), respectively. Median rod intercept time was 14.1 minutes (range, 4.4-20.0). Eyes with advanced disease based on retinal structure had significantly worse retinal function for several testing modalities. CONCLUSIONS. PPS maculopathy causes considerable visual function degradation that is not fully captured with BCVA testing. There was good correlation between other measures of visual function and disease severity. These findings deepen our concern regarding this patient safety issue.

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“…●IC/BPS: Most first‐ and second‐line treatments can be conducted via telemedicine including: patient education; self‐care counseling and behavioral modification (including diet changes); stress management techniques; and multimodal pain management using amitriptyline, cimetidine, hydroxyzine, or pentosan polysulfate 64 . Caution should be used when prescribing pentosan polysulfate as long‐term use has recently been associated with macular disease 65,66 . Self‐administered bladder instillations could be recommended for flares (EC). ●Dysmenorrhea and premenstrual syndrome: Most conservative treatment guidelines can be conducted via telemedicine, including lifestyle modifications (e.g.…”

Section: Resultsmentioning

confidence: 99%

Guidance for gynecologists utilizing telemedicine during COVID‐19 pandemic based on expert consensus and rapid literature reviews

Grimes

Balk

Dieter

et al. 2020

Intl J Gynecology & Obste

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Background: COVID-19 has impacted delivery of outpatient gynecology and shifted care toward use of telemedicine. Objective: To rapidly review literature and society guidelines and create expert consensus to provide guidance regarding management of outpatient gynecology scenarios via telemedicine. Search strategy: Searches were conducted in Medline and Cochrane databases from inception through April 15, 2020. Selection criteria: Literature searches were conducted for articles on telemedicine and abnormal uterine bleeding, chronic pelvic pain, endometriosis, vaginitis, and postoperative care. Searches were restricted to available English language publications. Data collection and analysis: Expedited literature review methodology was followed and 10 943 citations were single-screened. Full-text articles and relevant guidelines were reviewed and narrative summaries developed. Main results: Fifty-one studies on the use of telemedicine in gynecology were found. Findings were reported for these studies and combined with society guidelines and expert consensus on four topics (abnormal uterine bleeding, chronic pelvic pain and endometriosis, vaginal discharge, and postoperative care). Conclusions: Guidance for treating gynecological conditions via telemedicine based on expedited literature review, review of society recommendations, and expert consensus is presented. Due to minimal evidence surrounding telemedicine and gynecology, a final consensus document is presented here that can be efficiently used in a clinical setting.

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Phenotypic Spectrum of Pentosan Polysulfate Sodium–Associated Maculopathy

Cited by 93 publications

References 13 publications

Identification of Patients with Pentosan Polysulfate Sodium-Associated Maculopathy through Screening of the Electronic Medical Record at an Academic Center

Identification of Patients with Pentosan Polysulfate Sodium-Associated Maculopathy through Screening of the Electronic Medical Record at an Academic Center

Visual Function in Pentosan Polysulfate Sodium Maculopathy

Guidance for gynecologists utilizing telemedicine during COVID‐19 pandemic based on expert consensus and rapid literature reviews

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