Primary Sclerosing Cholangitis

• What is Primary Sclerosing Cholangitis?
• Statistics on Primary Sclerosing Cholangitis
• Risk Factors for Primary Sclerosing Cholangitis
• Progression of Primary Sclerosing Cholangitis
• How is Primary Sclerosing Cholangitis Diagnosed?
• Prognosis of Primary Sclerosing Cholangitis
• How is Primary Sclerosing Cholangitis Treated?
• Primary Sclerosing Cholangitis References

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

1. Braunwald, Fauci, Kasper, Hauser, Longo, Jameson. Harrison's Principles of Internal Medicine. 15th Edition. McGraw-Hill. 2001
2. Cotran, Kumar, Collins 6th edition. Robbins Pathologic Basis of Disease. WB Saunders Company. 1999.
3. Haslet C, Chiliers ER, Boon NA, Colledge NR. Principles and Practice of Medicine. Churchill Livingstone 2002.
4. Hurst JW (Editor-in-chief). Medicine for the practicing physician. 4th edition Appleton and Lange 1996.
5. Kumar P, Clark M. CLINICAL MEDICINE. WB Saunders 2002
6. Longmore M, Wilkinson I, Torok E. OXFORD HANDBOOK OF CLINICAL MEDICINE. Oxford Universtiy Press. 2001
7. McLatchie G and LEaper DJ (editors). Oxford Handbook of Clinical Surgery 2nd Edition. Oxford University Press 2002.
8. MEDLINE Plus
9. Raftery AT Churchill's pocketbook of Surgery. Churchill Livingsone 2001.
10. Tjandra, JJ, Clunie GJ, Thomas, RJS,; Textbook of Surgery, 2nd Ed, Blackwell Science, Asia. 2001.

Treatments Used in This Disease:

• Liver Transplants

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Article Dates:

Modified: 15/6/2011

Created: 20/9/2003