Primary sclerosing cholangitis is inflammation with progressive scarring and narrowing of the bile ducts in and outside the liver. Eventually, the ducts become blocked and then obliterated. Cirrhosis, liver failure, and sometimes bile duct cancer develop.
In primary sclerosing cholangitis, scarring worsens, eventually becoming severe (cirrhosis). The scar tissue narrows and blocks the bile ducts. As a result, bile salts, which help the body absorb fats, are not secreted normally. The disorder resembles primary biliary cirrhosis (see Primary Biliary Cirrhosis) except that it affects the bile ducts outside the liver as well as those in the liver. The cause is not known but is likely to be autoimmune (when the immune system attacks the body's own tissues—see Autoimmune Disorders).
Primary sclerosing cholangitis most often affects young men at an average age of 40. It commonly occurs in people with inflammatory bowel disease, especially ulcerative colitis. It tends to occur in families, suggesting that genes may be involved. An infection or injury of the bile ducts may trigger the disorder in people who have genes that make them susceptible to the disorder. The bile ducts may be injured during an endoscopic procedure, such as placement of tubes (stents) intended to keep the bile ducts open.
Symptoms usually begin gradually with worsening fatigue and itchiness. Jaundice (yellowish discoloration of the skin and whites of the eyes) tends to develop later.
Inflammation and recurring infection of the bile ducts (bacterial cholangitis) sometimes occur. Bacterial cholangitis causes attacks of pain in the upper abdomen, jaundice, and fever.
Because bile salts are not secreted normally, people may be unable to absorb enough fats and fat-soluble vitamins (A, D, E, and K). Such impaired bile secretion results in osteoporosis, easy bruising and bleeding, and stools that are greasy and foul-smelling (steatorrhea). Gallstones and bile duct stones develop in about three fourths of people with primary sclerosing cholangitis. The liver and spleen may enlarge.
As the disorder progresses, symptoms of cirrhosis (see Symptoms) develop. Advanced cirrhosis causes the following:
Some people have no symptoms until the disorder is advanced and cirrhosis is present. Symptoms may not appear for up to 10 years.
Cancer of the bile ducts (cholangiocarcinoma) develops in 10 to 15% of people with primary sclerosing cholangitis.
Usually, primary sclerosing cholangitis worsens gradually. Liver failure occurs about 12 years after the disorder is diagnosed.
The disorder may be suspected when results of liver function tests, done as part of an annual physical examination or for some unrelated reason, are abnormal. Then, ultrasonography is typically done first to check for blockage of bile ducts outside the liver. Tests that can confirm the diagnosis include the following:
Blood tests and ERCP may be done regularly to check for cancer of the bile ducts.
If people have no symptoms, no treatment is required. But twice a year, they need to have a physical examination and blood tests to monitor the progression of the disorder.
The drug ursodeoxycholic acid may help relieve itching. Recurring bacterial cholangitis is treated with antibiotics. ERCP is done as needed to widen (dilate) the blocked ducts. Sometimes tubes to keep the ducts open (stents) are inserted temporarily.
Liver transplantation (see Liver Transplantation) is the only treatment that prolongs life. It can cure some types of this otherwise fatal disorder. People with cirrhosis that causes serious complications or those with recurrent bacterial cholangitis may require liver transplantation.
If cancer of the bile ducts develops and surgery to remove the cancer is not possible, stents may be passed through an endoscope and placed in bile ducts that are blocked by the cancer. These stents open the ducts.
Last full review/revision February 2014 by Ali A. Siddiqui, MD