Primary sclerosing cholangitis

Clinical information

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.

Diagnostics

Cholangiography is the most important method to achieve a reliable diagnosis. Although a number of autoantibodies occur in PSC, it is not a typical autoimmune disease. However, it may be associated with autoimmune phenomena in childhood, including high titers of autoantibodies (e.g. ANA, ANCA and ASMA). In suspected cases of overlap syndrome with autoimmune hepatitis or primary biliary cholangitis (up to 10% of cases), serological antibody tests may provide additional diagnostic information.

Files

Autoimmune liver diseases

Differentiated ANCA determination using IIFT Mosaics