Primary sclerosing cholangitis (PSC) is a chronic choleastic liver disease with progressive destruction of the bile ducts. PSC has a prevalence of approximately 8 to 14 patients in 100,000 people. Men are more frequently affected by the disease than women (60-80%) and the disease is mainly diagnosed at the age of 30 to 60. PSC occurs more often in patients with chronic inflammatory bowel diseases (85 to 90% ulcerative colitis and 10 to 15%  Crohn’s disease). Most patients with PSC exhibit autoantibodies against granulocytes (pANCA) in the serological test; the main target antigen is DNA-bound lactoferrin.