Primary sclerosing cholangitis – a comprehensive review

Abstract

Primary sclerosing cholangitis (PSC) is a rare disorder characterised by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. Most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and a lack of proven surveillance strategies, patients currently have significant unmet needs. In the present seminar, we provide a comprehensive review of the status of the field. We emphasise developments related to patient stratification and disease behaviour, and provide an overview of management options from a practical, patient-centered perspective. We survey advances made in the understanding of PSC pathogenesis and summarise the ongoing efforts to develop an effective therapy based on these insights.

Consistent alterations in faecal microbiomes of patients with primary sclerosing cholangitis independent of associated colitis

“…1 There is no medical treatment available and patients have an increased risk of both hepatobiliary and bowel malignancies. 1 There is no medical treatment available and patients have an increased risk of both hepatobiliary and bowel malignancies.…”

“…2,3 As a result, most patients decease or receive liver transplantation 15-20 years after diagnosis. 1,[5][6][7] It has been postulated that PSC-associated colitis (PSC-IBD) represents a distinct IBD entity alongside Crohn's disease and UC. Cooccurrence of inflammatory bowel disease (IBD), typically in form of a mild pancolitis, has been reported in up to 80% of patients with PSC, and at least 2%-7.5% of IBD-patients develop PSC.…”

Incidence and outcome of colorectal cancer in liver transplant recipients: A national, multicentre analysis on 8115 patients

“…Patients with PSC/UC have been reported to have an increased risk of CRC development, potentially with a greater risk after LT due to a possible contribution of other factors such as the immunosuppressive therapy or a more aggressive course of the IBD after transplant …”

“…According to the European Liver Transplant Register, primary sclerosing cholangitis (PSC) is the primary indication for LT in approximately 5% of all adult recipients and is associated with inflammatory bowel disease (IBD) in up to 70% of patients . In comparison with the general population, patients with PSC‐IBD have a 10‐fold increased risk of CRC, while patients with PSC alone have a 5‐fold increased risk of CRC . Moreover, CRC in this group of patients appears to develop at a younger age and is diagnosed at a more advanced stage compared with the general population is predominantly localised in the ascending colon and represents one of the leading causes of death …”

Circulating markers of gut barrier function associated with disease severity in primary sclerosing cholangitis

“…Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease affecting intra‐ and extrahepatic bile ducts, eventually progressing to end‐stage liver disease . There is currently no medical treatment available.…”