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Controversies in the management of primary sclerosing cholangitis.

ABSTRACT: Primary sclerosing cholangitis (PSC) remains a rare but significant disease, which affects mainly young males in association with inflammatory bowel disease. There have been few advances in the understanding of the pathogenesis of the condition and no therapeutics with proven mortality benefit aside from liver transplantation. There remain areas of controversy in the management of PSC which include the differentiation from other cholangiopathies, in particular immunoglobulin G4 related sclerosing cholangitis, the management of dominant biliary strictures, and the role of ursodeoxycholic acid. In addition, the timing of liver transplantation in PSC remains difficult to predict with standard liver severity scores. In this review, we address these controversies and highlight the latest evidence base in the management of PSC.

SUBMITTER: Nayagam JS

PROVIDER: S-EPMC4757649 | biostudies-literature | 2016 Feb

REPOSITORIES: biostudies-literature

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Controversies in the management of primary sclerosing cholangitis.

Nayagam Jeremy S JS Pereira Stephen P SP Devlin John J Harrison Phillip M PM Joshi Deepak D

World journal of hepatology 20160201 5

Primary sclerosing cholangitis (PSC) remains a rare but significant disease, which affects mainly young males in association with inflammatory bowel disease. There have been few advances in the understanding of the pathogenesis of the condition and no therapeutics with proven mortality benefit aside from liver transplantation. There remain areas of controversy in the management of PSC which include the differentiation from other cholangiopathies, in particular immunoglobulin G4 related sclerosin ...[more]

PMID: 26925200

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Project description:Background & aimsData on the management of primary sclerosing cholangitis (PSC) in European expert centres are sparse. In this study, a PSC group from the ERN RARE-LIVER surveyed European hepatologists to uncover differences in real-life clinical practices.MethodsIn April 2020 a survey questionnaire was sent to members of the International PSC Study Group and ERN RARE-LIVER. Participants were asked about the size of their PSC cohort, use of medical treatments including ursodeoxycholic acid (UDCA) and surveillance for cholangiocarcinoma, gallbladder polyps and inflammatory bowel disease (IBD). Data were presented descriptively.ResultsEighty-two of 278 members responded. Fifty percent of physicians prescribed UDCA routinely to all their patients with PSC, whereas 12% never prescribed UDCA. UDCA was used for one or more indications including: alkaline phosphatase >1.5x the upper limit of normal, severe PSC changes, pruritus, PSC-IBD or patient demand. Few physicians offered other medical treatments than UDCA. The use of medical treatments was generally comparable in small (<99 patients) and large (≥99 patients) cohorts, as well as for adult and paediatric physicians. Most physicians routinely screened for cholangiocarcinoma and the most frequent modalities used were MRI and ultrasound. At detection of a gallbladder polyp of 6 mm, 46% of physicians recommended repeated ultrasound after 3-6 months, whereas 44% of physicians recommended immediate cholecystectomy. In patients with PSC without IBD at PSC diagnosis, 68% of physicians repeated colonoscopy within 3-5 years whereas 27% referred only patients who developed symptoms of IBD.ConclusionSubstantial variations in treatment and monitoring of European patients with PSC were discovered. Harmonisation of strategies is desirable to enable improved interpretation of outcome data and to optimise clinical patient care.Lay summaryIn this study, we explored how different centres in Europe manage primary sclerosing cholangitis (PSC), a rare inflammatory disease of the bile ducts. We collected information through a questionnaire sent to specialist physicians who were part of a European network for rare liver diseases. We found several differences in how patients with PSC were monitored and treated. This includes differences in surveillance for bile duct cancer, gallbladder polyps and inflammatory bowel disease. By pointing out these differences, we hope that management of PSC will be standardized, which could aid clinical research and benefit patients.

Dataset Information

Controversies in the management of primary sclerosing cholangitis.

Publications

Controversies in the management of primary sclerosing cholangitis.

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