This study is a biobank of specimens and clinical data for use in current and future research to better understand the cholestatic liver diseases primary biliary cirrhosis/cholangitis (PBC) and primary sclerosing cholangitis (PSC).

This study is a biobank of specimens and clinical data for use in current and future research to better understand the cholestatic liver diseases primary biliary cirrhosis/cholangitis (PBC) and primary sclerosing cholangitis (PSC).

Blood, saliva, stool, and urine samples will be collected periodically during patient visits, mail-home kit (collected locally), or via remote phlebotomy at a place directed by the subject. Bile and bile duct cells will be collected during clinically necessary Endoscopic Retrograde Cholangiopancreatography (ERCP) procedures being performed at Mayo Clinic for clinical reasons. Anticipated research will focus on multi-omics assessments of biospecimens to better define how these diseases start and progress in order to develop novel tests for early detection of complication and better disease prognostication.

Inclusion Criteria:

PSC

• Patients diagnosed with PSC and who are between the age of 18 and 85 at time of enrollment in the study.
• The diagnosis of PSC will be based on standard PSC criteria including clinical and biochemical evidence of chronic cholestasis of at least six months duration, positive cholangiographic findings, and compatible liver biopsies if available.
• Patients with PSC who have undergone orthotopic liver transplantation will be offered enrollment except for collection of bile - please see below under Exclusion Criteria.
• Women with PSC of childbearing potential and pregnant women will be offered enrollment because there is no risk to an unborn child in this investigation.

PBC

• Patients diagnosed with PBC and who are between the age of 18 and 85 at time of enrollment in the study.
• The diagnosis of PBC will be based on standard PBC criteria including clinical and biochemical evidence of chronic cholestasis of at least six month duration, positive anti-mitochondrial antibodies in serum and compatible liver biopsies, if available.
• Patients with PBC who have undergone orthotopic liver transplantation will be offered enrollment except for collection of bile - please see below under Exclusion Criteria.
• Women with PBC of childbearing potential and pregnant women will be offered enrollment because there is no risk to an unborn child in this investigation.

Controls

• Controls without history of PBC, PSC, or evidence of other chronic liver disease of either gender that participate in this study will be between the ages of 18 and 85.

Liver Disease Controls

• Patients without history of PBC or PSC but do have evidence of other chronic liver disease of either gender will be offered participation in this study if between the ages of 18 and 85.

Exclusion Criteria (all subjects):

• PBC or PSC patients with known and overlapping other chronic liver diseases
• Patients unable to provide informed consent
• Prisoners and institutionalized individuals

Exclusion Criteria (for bile collection during ERCP)

• PSC with orthotopic liver transplantation
• History of Roux en Y

Patients with PBC, PSC, controls without liver disease, and with other chronic liver disease without history of PBC or PSC seen or followed at Mayo Clinic will be invited to participate in this study.