Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder of unknown causation that is frequently associated with inflammatory bowel disease (IBD). PSC is characterized by diffuse inflammation and fibrosis of the biliary tree and usually leads to biliary cirrhosis, which can be complicated by portal hypertension and liver failure.
Before the widespread availability of endoscopic retrograde cholangiopancreatography (ERCP) in the late 1970s, PSC was considered a rare disease. It is now seen as an important cause of chronic cholestasis in adults and is increasingly diagnosed in the pediatric population. It is unclear whether the prevalence of the disease has increased, but emerging data suggest that it has. The recognized association of PSC and IBD and the common screening of IBD patients with liver enzymes have also probably increased the frequency with which the diagnosis of PSC is made. This greater recognition of the disease and increased experience have led to greater understanding of the course of the disease, although the cause and identification of specific beneficial therapies have eluded investigators so far.
The etiology of PSC has remained poorly understood since the earliest description of the disease. The current thinking is that PSC occurs as a consequence of a genetically determined dysregulated immune system, resulting in an uncontrolled inflammatory response in the bile ducts with destruction and fibrosis and, ultimately, biliary cirrhosis. The allo- and/or autoantigen(s) that trigger this restricted inflammatory response in the bile ducts are unknown. Putative agents include bacterial antigens absorbed through a diseased bowel mucosa, particularly in patients with underlying IBD, as well as cytotoxic bile acids, viral infections, and ischemic injury.
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