What is Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis is a disease affecting the bile ducts within and outside the liver. There is inflammation, fibrosis and strictures of the intra and extra-hepatic bile ducts.

Statistics on Primary Sclerosing Cholangitis

This disease is not very common -7/100,000 is estimated to be the prevalence. The disease is seven times more common in men than women (70% of patients affected are men). The usual age of presentation is around 40 years of age.

Risk Factors for Primary Sclerosing Cholangitis

Predisposing factors include being of the male sex, having a history of inflammatory bowel disease, and AIDS.

Progression of Primary Sclerosing Cholangitis

This condition is a progressive liver failure that leads to biliary cirrhosis and ultimately liver failure. The survival of patients whether symptomatic or asymptomatic is reported to be below the normal population. There are currently no treatments that halt disease progression.

How is Primary Sclerosing Cholangitis Diagnosed?

  • Liver function tests: cholestasis (often show raised bilirubin, ALP and GGT with modest elevation of ALT and AST).
  • Coagulation profile – prolonged clotting times.
  • Full blood count– thrombocytopaenia (low platelety)may be found secondary to the portal hypertension (splenomegaly). May be a normocytic normochromic anaemia due to concomitant gastrointestinal blood loss secondary to inflammatory bowel disease.

Prognosis of Primary Sclerosing Cholangitis

Average duration to fulminant liver failure can range between 9 and 12 years. Several features correlate with a poor prognosis. These include: an enlarged liver or spleen, anaemia, cirrhosis, female sex and older age. Increasing bilirubin also correlates with disease progression. In one study of hyperbilirubinaemic patients, the survival of patients whether symptomatic or asymptomatic is reported to be below the normal population.

How is Primary Sclerosing Cholangitis Treated?

Medications so far have not been effective in slowing or preventing the progression of the disease. Symptom control is the mainstay of management. A liver transplantation is considered for patients with a more advanced disease, a transplantation has a 90% survival rate over years.

Primary Sclerosing Cholangitis References

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