Primary sclerosing cholangitis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Signs and symptoms
Diagnosis
Etiology
Pathophysiology
Bile ducts, both intra- and extrahepatically (inside the liver and outside), are inflamed and develop scarring, obstructing the flow of bile. As bile assists in the enteric breakdown and absorption of fat, the absence of bile leads to fat malabsorption. The bile accumulates in the duct, leading to liver cell damage and liver failure.
Epidemiology
It is more prevalent in men than in women. The disease normally starts from age 30 to 60. It can however also start with children. PSC progresses slowly, so the disease can be active for a long time before it is noticed or diagnosed.
Screening
PSC is associated with cholangiocarcinoma, which are tumors involving the biliary tree. Screening for cholangiocarcinoma in patients with PSC is encouraged, but there is no general consensus on the modality and interval of choice.
Therapy
Standard treatment includes ursodiol, a bile acid naturally produced by the liver, which has been shown to lower elevated liver enzyme numbers in people with PSC, but has not yet been proven effective at prolonging the life of the liver. Treatment also includes medication to relieve itching (antipruritics) and bile acid sequesterants (cholestyramine), antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamin A, vitamin D, and vitamin K.
In some cases, ERCP, which may involve stenting of the common bile duct, may be necessary in order to open major blockages (dominant strictures).
Liver transplantation (including live transplants whereby a portion of a living donor is given to the recipient) is an option if the liver begins to fail.
See also
- Bilirubin
- Child-Pugh score
- Cholangiocarcinoma
- Liver function tests
- Primary biliary cirrhosis
- Alagille syndrome
- Progressive familial intrahepatic cholestasis
- Walter Payton (died of primary sclerosing cholangitis)
External links
- Additional Literature about PSC
- Link to PSC Partners, a nonprofit foundation for PSC patients and caregivers
- PSC Trust is a medical research charity
- Liver Families, a pediatric liver support group
- The Morgan Foundation for the Study of PSC