Natural History and Outcomes of Primary Sclerosing Cholangitis with and without Dominant Stricture: A prospective study
Lead Investigator e-mail:
A/Prof Ammar Majeed
Primary sclerosing cholangitis (PSC) is a chronic, idiopathic cholestatic liver disease. It is characterized by progressive biliary inflammation and fibrosis resulting in multifocal biliary strictures. Despite the absence of effective therapy, the natural history of PSC is variable with cirrhosis, hepatic failure, and cholangiocarcinoma noted but not invariable sequelae. While PSC has been studied in a number of different cohorts around the world, there is no published data in an Australian setting.
The study plans to recruited any patient with PSC and follow them prospectively to better understand the natural history of PSC and the predictors of development of liver related outcomes as well as overall survival.
The main aims of the study include describing the natural history of PSC, including the development of biliary and non-biliary malignancies, and end-stage liver disease and the need for liver transplantation in a prospective well characterized cohort of patients with PSC in Australia. To compare survival in PSC patients with and without dominant stricture. To investigate predictors of outcome in PSC patients including the influence of demographic factors, ethnicity, treatment with UDCA, IBD and its treatment on the course of PSC. To study the influence of gut microbiota on liver and non-liver related PSC outcomes. And to assess genetic and exploring biomarkers in PSC, looking at specific candidates, and using proteomics to identify potential predictors of disease outcomes.