Primary sclerosing cholangitis (PSC) is an idiopathic, inflammatory disorder of the bile ducts frequently associated with inflammatory bowel diseases (IBD) of the colon affecting both adults and children. The progression of liver fibrosis in PSC is slow and variable. In addition, PSC is associated with complications such as cholangiocarcinoma, gallbladder cancer, and colorectal adenocarcinoma which may occur at any stage of fibrosis. Defining the natural history of PSC is multiple populations, developing prognostic models, and validating biomarkers of disease progression are critical for the development of new therapies for the treatment of PSC.
The Natural History and Biomarkers Working Group is committed to:
- Developing retrospective/prospective databases using common data elements with representation of the spectrum of populations affected by PSC.
- Supporting Biomarker development including discovery and validation of established tests (i.e. vibration controlled transient elastography in the FICUS study) as well as novel biomarkers.
- Whole exome sequencing of PSC-associated biliary tract cancers (Trine Folseraas, Oslo)
- CCA Registry database (update: Jesus M. Banales, San Sebastian)
- Animal models of PSC-CCA (update: Luca Fabris, Padua)
- International validation study of new non-invasive protein biomarkers inserum extracellular vesicles of PSC, CCA, HCC patients (Jesus M. Banales, San Sebastian)
- Imaging of peribiliary glands and histo-morphology correlates in PSC-CCA. A multicentre international study proposal ( Cardinale, G. Carpino, Rome)
- Metabolomics and lipidomics in assessment of disease progression and risk for biliary dysplasia in PSC ( Farkkila, Helsinki)
- PSC to CCA – Which role does the stroma play? ( Mertens, Zurich)
- Role of PSC-related intestinal dysbiosis in colitis-associated colorectal cancer (Massimo C. Fantini, Rome)