Precision medicine in primary sclerosing cholangitis

Maurice, James; Thorburn, Douglas

doi:10.1111/1751-2980.12788

Cited by 6 publications

(5 citation statements)

References 71 publications

(84 reference statements)

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“…14,15 Grade Adjusted Hazard Ratio (HR) for combined end-points 0,1 0,2 0,5 The adapted Nakanuma classification, and now the PSC histoscore, bring additional value to the applicability of liver biopsy specimens. 41 The unique patient cohort and data available in the PSC registry enabled this comprehensive retrospective study. The follow-up periods were long, and the cohort consisted of patients at different stages of disease.…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, the European Medicines Agency (EMA) and U.S. Food and Drug Administration (FDA) highly recommend using the histological fibrosis stage and P‐ALP values as surrogate markers in drug trials 14,15 . The adapted Nakanuma classification, and now the PSC histoscore, bring additional value to the applicability of liver biopsy specimens 41 …”

Section: Discussionmentioning

confidence: 99%

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Novel histological scoring for predicting disease outcome in primary sclerosing cholangitis

et al. 2022

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Background Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease that may lead to liver cirrhosis or cholangiocarcinoma. Liver histology and fibrosis stage are predictive markers of disease progression, and histological cirrhosis is defined as a significant endpoint. PSC‐specific histological scoring methods are lacking at present. We aimed to develop a tailored classification system for PSC, the PSC histoscore, based on histological features associated with disease progression. Methods In total, 300 PSC patients diagnosed between 1988 and 2018 were enrolled; their data were collected from the PSC registry (Helsinki University Hospital), and liver specimens were obtained from the Biobank of Helsinki. Five histological features included in the adapted Nakanuma scoring system and three additional parameters typical for PSC histology were evaluated and compared with the clinical and laboratory data. A compound endpoint consisting of liver transplantation, development of cholangiocarcinoma, or death was used as outcome measurement. Results Stage (fibrosis, bile duct loss, ductular reaction, and chronic cholestasis) and grade (portal inflammation, portal edema, hepatitis activity, and cholangitis activity) parameters were found to be independent predictive risk factors for the compound endpoint (P < 0.001). High disease grade (2–6) and stage (2–4) better correlated with clinical endpoints when evaluated with the PSC histoscore system compared to the adapted Nakanuma classification. The risk for disease progression in sequential endoscopic retrograde cholangiography (ERC) examinations was increased with elevated total PSC histoscores. Conclusion The PSC histoscore is a novel histological classification system for PSC. Our findings support the applicability of liver histology as a marker for disease progression.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Novel histological scoring for predicting disease outcome in primary sclerosing cholangitis

et al. 2022

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“…Vancomycin is a glycopeptide antibiotic that also has an immunomodulatory effect due to the decrease in T cell cytokine production [104]. Vancomycin was compared to metronidazole [105] and to placebo [106] in two randomized trials in PSC patients with or without IBD, and a significant reduction in ALP levels and the Mayo score was reported.…”

Section: Modulation Of the Gut Microbiomementioning

confidence: 99%

The Management of Cholestatic Liver Diseases: Current Therapies and Emerging New Possibilities

Mazzetti

Marconi

Mancinelli

et al. 2021

JCM

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Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are two chronic cholestatic liver diseases affecting bile ducts that may progress to biliary cirrhosis. In the past few years, the increasing knowledge in the pathogenesis of both diseases led to a growing number of clinical trials and possible new targets for therapy. In this review, we provide an update on the treatments in clinical use and summarize the new drugs in trials for PBC and PSC patients. Farnesoid X Receptor (FXR) agonists and Pan-Peroxisome Proliferator-Activated Receptor (PPAR) agonists are the most promising agents and have shown promising results in both PBC and PSC. Fibroblast Growth Factor 19 (FGF19) analogues also showed good results, especially in PBC, while, although PBC and PSC are autoimmune diseases, immunosuppressive drugs had disappointing effects. Since the gut microbiome could have a potential role in the pathogenesis of PSC, recent research focused on molecules that could change the microbiome, with good results. The near future of the medical management of these diseases may include new treatments or a combination of multiple drugs targeting different signaling pathways at different stages of the diseases.

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“…At the same time, 5% of patients with IBD are likely to be diagnosed with PSC as well, though, PSC is not related to the IBD course. [ 72 ] The incidence of osteoporosis with PSC has been reported at 4%–10%, and correlated with duration of IBD, low body mass index, and older age. [ 73 ] In late stage disease, fat-soluble vitamin deficiency, and steatorrhea are more likely to take place due to chronic cholestasis.…”

Section: Primary Sclerosing Cholangitismentioning

confidence: 99%

Cholestatic liver disease

Mawardi

Alalwan

Fallatah

et al. 2021

Saudi Journal of Gastroenterology

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Cholestatic liver diseases (CLDs) are a group of diseases characterized by jaundice and cholestasis as the main presentation with different complications, which have considerable impact on the liver and can lead to end-stage liver disease, cirrhosis, and liver-related complications. In the last few years, tremendous progress has been made in understanding the pathophysiology, diagnosis, and treatment of patients with these conditions. However, several aspects related to the management of CLDs remain deficient and unclear. Due to the lack of recommendations that can help in the management, treatment of those conditions, the Saudi Association for the Study of Liver diseases and Transplantation (SASLT) has created a task force group to develop guidelines related to CLDs management in order to provide a standard of care for patients in need. These guidelines provide general guidance for health care professionals to optimize medical care for patients with CLDs for both adult and pediatric populations, in association with clinical judgments to be considered on a case-by-case basis. These guidelines describe common CLDs in Saudi Arabia, with recommendations on the best approach for diagnosis and management of different diseases based on the Grading of Recommendation Assessment (GRADE), combined with a level of evidence available in the literature.

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Precision medicine in primary sclerosing cholangitis

Cited by 6 publications

References 71 publications

Novel histological scoring for predicting disease outcome in primary sclerosing cholangitis

Novel histological scoring for predicting disease outcome in primary sclerosing cholangitis

The Management of Cholestatic Liver Diseases: Current Therapies and Emerging New Possibilities

Cholestatic liver disease

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