Inclusion Criteria:

PSC

• Patients diagnosed with PSC between the age of 18 and 85.
• 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 between the age of 18 and 85.
• The diagnosis of PBC will be based on standard PBC criteria including clinical and biochemical evidence of chronic cholestasis of at least six months duration, positive anti-mitochondrial antibodies (>1:40 titer) in serum and compatible liver biopsies, if available6.
• Patients with PBC who have undergone orthotopic liver transplantation will be offered enrollment.
• 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 will participate in this study should be between the ages of 18-85. 

Liver Disease Controls

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

Exclusion Criteria (all subjects):

• PBC or PSC patients that overlap with other chronic liver diseases.
• Patients unable to provide inform consent.
• Prisoners and institutionalized individuals.
• Controls invited/consented through the Mayo Clinic Biobank are not eligible if they have any of the following conditions:
  • Inflammatory bowel disease (IBD);
  • Colitis;
  • Colectomy;
  • History of cancer (including skin cancers(only melanoma));
  • History of radiation treatments or chemotherapy;
  • History of organ transplant.

Exclusion Criteria (for bile collection during ERCP):

• PSC with orthotopicliver transplantation.