Introduction: It is currently assumed that interstitial cystitis/bladder pain syndrome is caused by damage to the glycosaminoglycane layer on the urothelium of the urinary bladder. This layer can be repaired by oral therapy with pentosan polysulfate sodium. The focus of this article is on the history of this drug, its efficacy, its valuation in guidelines and especially the possible correlation with maculopathy.
Methods: Literature research in PubMed and Embase.
Results: PPS has a US and European approval for the therapy of IC characterised by glomerulations or a Hunner lesion detected by endoscopy and bladder distension. Its efficacy was proven in randomised trials. This led to a recommendation as a basic pharmaceutical therapy (in addition to behavioural intervention, dietary therapy or other drug treatments such as pain therapy). After a treatment period of six months, efficacy should be re-evaluated. Side-effects include mild haemodilution, nausea and loss of hair. Two publications of a US eye clinic have recently postulated a correlation between prolonged high-dose therapy with PPS and a special kind of maculopathy. Although this correlation was rejected in a large-scale health service study in Germany, a "red-hand-letter" led to the recommendation to perform an ophthalmologic examination before and during the treatment with PPS. Due to a pending litigation between payers and the distributor, PPS is currently out of trade in Germany. However, PPS can still be prescribed but must be imported from adjacent European countries. Unfortunately, these modalities have led to a significant undersupply of patients with IC/BPS. It is feared that this undersupply will increase further as the litigation is ongoing.
Conclusion: Being the only causally acting compound in the therapy of IC/BPS, PPS has an exceptional status. Although an ongoing litigation is pending in Germany and the correlation with maculopathy is still unclear, PPS must remain part of the current and future therapy of IC/BPS.
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http://dx.doi.org/10.1055/a-1629-0199 | DOI Listing |
Investig Clin Urol
January 2025
Department of Urology, Ewha Womans University College of Medicine, Seoul, Korea.
Purpose: Although the mechanism underlying interstitial cystitis/bladder pain syndrome (IC/BPS) remains unclear, oxidative stress is suggested to be implicated in IC/BPS development. Sea buckthorn (SB; L.) contains several compounds with antioxidant properties.
View Article and Find Full Text PDFPLoS One
January 2025
Verana Health, San Francisco, CA, United States of America.
Purpose: To evaluate prevalence and incidence rates of pigmentary maculopathy and retinopathy (PM/PR), and visual acuity (VA) changes in patients exposed to pentosan polysulfate sodium (PPS) and in patients with interstitial cystitis (IC) not exposed to PPS.
Methods: This is a retrospective cohort study (January 2015-March 2021) which included adult de-identified patients from the American Academy of Ophthalmology IRIS® Registry (Intelligent Research in Sight) and Komodo Health database. Three patient cohorts were identified: two PPS-exposed patient cohorts, and Non-PPS-exposed IC patient cohort.
Sci Rep
January 2025
Xiamen University Affiliated Xiamen Eye Center, Fujian Provincial Key Laboratory of Ophthalmology and Visual Science, Fujian Engineering and Research Center of Eye Regenerative Medicine, Eye Institute of Xiamen University, School of Medicine, Xiamen University, Xiamen, 361102, Fujian, China.
Macular degeneration is a leading cause of irreversible vision loss, significantly impacting quality of life. To enhance clinical practice and reduce the risk of drug-related macular degeneration, we analyzed drug-related trends using real-world data. Disproportionality analysis of adverse event reports from the FDA Adverse Event Reporting System (FAERS, 2004-2023) identified 67,683 cases involving 1402 drugs.
View Article and Find Full Text PDFAm J Ophthalmol Case Rep
December 2024
Shiley Eye Institute, University of California, San Diego, La Jolla, CA, USA.
Purpose: To report a case of pentosan polysulfate sodium (PPS) maculopathy with cystoid macular edema (CME) with relatively low cumulative PPS exposure and a history of concurrent hydroxychloroquine use.
Observations: A 46-year-old female was treated with PPS daily for 10 years, and hydroxychloroquine intermittently over a span of five years, actively taking hydroxychloroquine for a sum of three years during PPS therapy. Despite a low risk for toxicity based on the cumulative exposure to either medication, fundoscopic examination and multimodal imaging revealed severe PPS maculopathy with CME two and a half years post-PPS cessation.
Surv Ophthalmol
December 2024
Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, CA, United States; Greater Los Angeles VA Healthcare Center (D.S.), Los Angeles, CA, USA. Electronic address:
Pentosan polysulfate sodium (PPS) maculopathy is a recently described condition of macular toxicity. We report the prevalence (15 %), the clinical characteristics, and the multimodal imaging (MMI) features of PPS maculopathy based on a final prospective analysis of a cohort of 154 subjects and highlight the association with inflammatory bowel disease (IBD). In addition, we summarize the most recent and relevant literature on PPS maculopathy.
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