A Novel Multimethod Image Analysis to Quantify Pentosan Polysulfate Sodium Retinal Toxicity

Hadad, Aviel; helmy, omar; Leeman, Samuel; Schaal, Shlomit

doi:10.1016/j.ophtha.2019.10.013

Cited by 12 publications

(12 citation statements)

References 4 publications

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“…Such diagnoses were also noted in the prior study by Hanif et al 8 Imaging analyses reported by Hadad et al showed that all patients (n=17) using 100mg of PPS daily for at least 3 years had mottling changes of the retina on near-infrared imaging, 75% of patients showed a hypoautofluorescence defect in the macula similar to those reported by Pearce et al, 4 and hyperreflectivity, thickening of the foci of the RPE, and a flying saucer macular sign were also noted on OCT imaging. 14 A recent prospective study by Wang et al found that patients with a cumulative dosage over 1500g had a significant risk of developing a PPS-associated maculopathy. 6 They recommend baseline ophthalmological examinations with multimodal imaging of patients who are to receive PPS in a cumulative dose of about 500g.…”

Section: Discussionmentioning

confidence: 99%

Maculopathy Secondary to Pentosan Polysulfate Use: A Single-Center Experience

Kalbag¹,

Maganti²,

Lyon³

et al. 2021

OPTH

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Aim: To investigate the prevalence of retinal pathology in patients with a history of exposure to pentosan polysulfate sodium (PPS). Methods: Patients exposed to PPS and seen in the ophthalmology clinic at Northwestern University during 1/1/2002 to 1/1/2019 were identified from electronic health records (EHR) by an electronic data warehouse (EDW) search. Visual acuity (VA), reasons for clinic visit, ocular conditions, and duration of exposure to PPS were noted. Chart review was performed for fundus exam findings and ophthalmologic imaging, specifically fundus photography, fundus autofluorescence, and ocular coherence tomography (OCT) images. When OCT or fundus photography was available, studies were evaluated by two independent graders. Results: A total of 131 patients who were exposed to PPS and seen at the Northwestern Ophthalmology clinic were identified in the EHR. Forty patients of 131 had imaging. Patients with imaging or fundus examination suspicious for PPS maculopathy were placed into the suspect group. Of the 40 patients that had imaging, 5 (12.5%) had features suspicious for PPS maculopathy. Of the remaining 91, 5 (5.4%) had macular pigmentary changes described on fundus exam. Among the 10 patients in the suspect group, the average duration of PPS use was 4.2 years (range 0.3-11.6 years, interquartile range 5.5 years) and the average cumulative dose was 380g (range 29-1092g, interquartile range 132g). Conclusion: A novel drug-induced maculopathy has been associated with PPS use with a distinct clinical constellation that can be accurately identified with multimodal imaging.

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Section: Discussionmentioning

confidence: 99%

Maculopathy Secondary to Pentosan Polysulfate Use: A Single-Center Experience

Kalbag¹,

Maganti²,

Lyon³

et al. 2021

OPTH

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“…So far it remains unclear whether there is a minimum safe cumulative dose, as 1 case of the disease presented in a 44-year-old woman who had taken only 435 g of pentosan total 28 . It is also unclear to what extent cumulative dose per unit body weight affects the development of maculopathy, with 1 study showing a higher risk with a dose greater than 20 g/kg 29 . Daily dose may modulate risk as well.…”

Section: Pentosan Maculopathymentioning

confidence: 99%

“…28 It is also unclear to what extent cumulative dose per unit body weight affects the development of maculopathy, with 1 study showing a higher risk with a dose greater than 20 g/kg. 29 Daily dose may modulate risk as well. Wang and coworkers 25 found that affected patients took a mean daily dose of 444.8 mg versus 301.8 mg for unaffected patients.…”

Section: Associations Risk Factors and Prevalencementioning

confidence: 99%

Age-Related Macular Degeneration Masquerade: A Review of Pentosan Polysulfate Maculopathy and Implications for Clinical Practice

Mukhopadhyay

Boyce

Gehrs

et al. 2022

Asia-Pacific Journal of Ophthalmology

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Pentosan polysulfate (PPS) sodium (Elmiron) is the only Food and Drug Administration (FDA)–approved oral medication to treat interstitial cystitis, also known as bladder pain syndrome. A symptomatic pigmentary maculopathy associated with PPS was reported in 2018. Since then, recognition of this unique drug toxicity has increased rapidly. This potentially sight-threatening side effect prompted the FDA in June 2020 to update the label for PPS to warn about “retinal pigmentary changes.” A challenging feature of pentosan maculopathy is its ability to mimic many other retinal conditions, including inherited retinal dystrophies such as pattern dystrophy, mitochondrially inherited diabetes and deafness, and Stargardt disease, and age-related macular degeneration. In this review, we discuss the history of PPS maculopathy and its implications for thousands of at-risk interstitial cystitis patients. We use published literature and an illustrative case from our institution to highlight the importance of diagnosing PPS maculopathy. We also compare PPS maculopathy to age-related macular degeneration, explain why differentiating between the 2 is clinically important, and highlight avenues for further research. Finally, we highlight the paucity of data on patients of color and why this lack of understanding may impact patient care.

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“…While toxic dose thresholds are still unclear, Wang et al noted no affected patient with cumulative doses <1500 gm, and in prior studies all affected patients except one reported at least a 500 gm cumulative dose. [207,208] A mathematical analysis of one cohort found the greatest correlation with standardized cumulative PPS dose adjusted to weight, [210] with 75% (n = 3) of patients with accumulated dose of >20 gm/kg demonstrating obvious hypo-AF defects on SW-AF. Prevalence of PPS-toxicity is so far reported as approximately 20%.…”

Section: Pentosan Maculopathymentioning

confidence: 99%

Fundus Autofluorescence and Clinical Applications

Pole

Ameri

2021

JOVR

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Fundus autofluorescence (FAF) has allowed in vivo mapping of retinal metabolic derangements and structural changes not possible with conventional color imaging. Incident light is absorbed by molecules in the fundus, which are excited and in turn emit photons of specific wavelengths that are captured and processed by a sensor to create a metabolic map of the fundus. Studies on the growing number of FAF platforms has shown each may be suited to certain clinical scenarios. Scanning laser ophthalmoscopes, fundus cameras, and modifications of these each have benefits and drawbacks that must be considered before and after imaging to properly interpret the images. Emerging clinical evidence has demonstrated the usefulness of FAF in diagnosis and management of an increasing number of chorioretinal conditions, such as agerelated macular degeneration, central serous chorioretinopathy, retinal drug toxicities, and inherited retinal degenerations such as retinitis pigmentosa and Stargardt disease. This article reviews commercial imaging platforms, imaging techniques, and clinical applications of FAF.

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A Novel Multimethod Image Analysis to Quantify Pentosan Polysulfate Sodium Retinal Toxicity

Cited by 12 publications

References 4 publications

Maculopathy Secondary to Pentosan Polysulfate Use: A Single-Center Experience

Maculopathy Secondary to Pentosan Polysulfate Use: A Single-Center Experience

Age-Related Macular Degeneration Masquerade: A Review of Pentosan Polysulfate Maculopathy and Implications for Clinical Practice

Fundus Autofluorescence and Clinical Applications

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