Sclerosing cholangitis refers to chronic cholestatic syndromes characterised by patchy inflammation, fibrosis, and destruction of the intrahepatic and extrahepatic bile ducts. Progression obliterates the bile ducts and leads to cirrhosis, liver failure, and sometimes cholangiocarcinoma.
The most common form of sclerosing cholangitis is primary sclerosing cholangitis (PSC). 70% of patients with PSC are men, with mean age at diagnosis 40 years. PSC has no known cause.
PSC is suspected in patients with unexplained abnormalities in liver biochemical tests, particularly in those with inflammatory bowel disease (IBD). A cholestatic pattern is typical: elevated alkaline phosphatase and gamma-glutamyltransferase (GGT) rather than aminotransferases. Gamma globulin level and IgM levels tend to be increased (30-50%). The only and nonspecific reactivity is an atypical perinuclear antineutrophil cytoplasmic antibody (“xANCA”). Characteristically, antimitochondrial antibodies (AMA), which are typically positive in primary biliary cirrhosis (PBC), are negative in PSC sera.
However, 80% of patients have inflammatory bowel disease (IBD), most often ulcerative colitis (UC). Other associated conditions include rheumatic connective tissue disorders, alloimmune disorders, and immunodeficiency syndromes.
The childhood disease called autoimmune sclerosing cholangitis (ASC) presents with serological features resembling autoimmune hepatitis type 1.