Primary Sclerosing Cholangitis: Causes

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