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- Primary sclerosing cholangitis (PSC) definition
- What causes primary sclerosing cholangitis?
- What are the risk factors for primary sclerosing cholangitis?
- What are the signs and symptoms of primary sclerosing cholangitis?
- How is the diagnosis of primary sclerosing cholangitis made?
- What is the treatment for primary sclerosing cholangitis?
- Liver transplant
- What complications are associated with primary sclerosing cholangitis?
- Can primary sclerosing cholangitis be prevented?
- What is the prognosis and life expectancy for a person with primary sclerosing cholangitis?
Liver transplant is the only "cure" for primary sclerosing cholangitis, but it is only recommended for patients whose disease has progressed to liver failure. The three-year U.S. survival rate for all transplants is 81%. The five-year survival rate is about 75%, according to the data from the Scientific Registry of Transplant Recipients.
The goal of liver transplantation is to restore liver function. Though unlikely, it is possible for PSC to recur in the new liver.
What complications are associated with primary sclerosing cholangitis?
PSC is a progressive disease, damaging the bile ducts and reducing the bile flow, ultimately leading to portal hypertension, cirrhosis, and liver failure.
Some cancers are associated with primary sclerosing cholangitis including gallbladder cancer, hepatocellular cancer (cancer of the liver cells) and cholangiocarinoma (cancer of the bile ducts). The combination of inflammatory bowel disease and PSC increases the risk of developing cancers of the colon and rectum
Can primary sclerosing cholangitis be prevented?
The specific cause of PSC is still unknown and therefore it seems not to be preventable. It is appropriate for patients with inflammatory bowel disease (especially ulcerative colitis) and their health care professional to be aware of the relationship with primary sclerosing cholangitis, in case symptoms suggestive of the disease arise. However, PSC is seen in only 3% of patients with inflammatory bowel disease.
What is the prognosis and life expectancy for a person with primary sclerosing cholangitis?
Aside from liver transplantation, there are no effective treatments for PSC. In the US, studies suggest that life expectancy ranges from 9 to 18 years if the patient does not undergo liver transplantation. Using different population study models, researchers in the Netherlands concluded that life expectancy may be longer than 21 years from the time when the diagnosis is made.
The prognosis and life expectance is poorer in older patients, those who have an enlarged liver and spleen, and in patients who are persistently jaundiced with elevated bilirubin levels in their bloodstream.
Medically reviewed by John A. Daller, MD; American Board of Surgery with subspecialty certification in surgical critical care
Boonstra K, et al. Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis. Hepatology 2013 Dec; 58(6):2045-55).
Razumilava N. et al. Cancer surveillance in patients with primary sclerosing cholangitis. Hepatology 2011 Nov; 54(5):1842-52.).