Pathogenesis (disease development)
The pathogenesis of primary sclerosing cholangitis (PSC) is as yet unknown. Immunologic factors, genetic associations (HLA associations), and a genetic background (familial clustering) are discussed.
PSC is a chronic cholestatic disease (biliary obstruction) that leads to progressive destruction of the intra- and/or extrahepatic (outside and inside the liver) biliary system.
Etiology (causes)
The etiology of primary sclerosing cholangitis is as yet unknown.
Biographic Causes
- Genetic burden
- By parents, grandparents (familial clustering).
- HLA-associated (HLA-B8, HLA-DR3, HLA-DRw52a).
Disease-related causes
- Autoimmune diseases