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.
Symptoms begin gradually and include worsening fatigue, itchiness, and, later, jaundice.
An imaging test can confirm the diagnosis.
Treatment focuses on relieving symptoms, but only liver transplantation can prolong life.
Bile is a fluid that is produced by the liver and aids in digestion. Bile is transported through small tubes (bile ducts) that carry bile through the liver and then from the liver to the gallbladder and to the small intestine. (See also Overview of Gallbladder and Bile Duct Disorders and figure View of the Liver and Gallbladder.)
In primary sclerosing cholangitis, bile ducts become inflamed, which can lead to scarring of the bile ducts and liver tissue that progresses, eventually becoming severe (a condition called 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 cholangitis 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).
Primary sclerosing cholangitis may affect the large and small bile ducts, may affect primarily the small bile ducts, or may share features with autoimmune hepatitis.
Primary sclerosing cholangitis most often affects young men and is most often diagnosed between the ages of 25-45 years. 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.
Symptoms of Primary Sclerosing Cholangitis
Symptoms usually begin gradually with worsening fatigue and itchiness. Jaundice (yellowish discoloration of the skin and whites of the eyes) tends to develop later. Many patients, though, have no symptoms and discover the disease based on abnormalities in blood testing done for other reasons.
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 (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 develop. Advanced cirrhosis causes the following:
Increased blood pressure in the vein that carries blood from the intestines to the liver (portal hypertension)
Accumulation of fluid in the abdominal cavity (ascites)
Liver failure, which can be fatal
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 20% of people with primary sclerosing cholangitis.
Usually, primary sclerosing cholangitis worsens gradually, resulting in liver failure.
Diagnosis of Primary Sclerosing Cholangitis
Liver blood tests
Ultrasound, followed by other imaging tests
Magnetic resonance cholangiopancreatography (MRCP)
Endoscopic retrograde cholangiopancreatography (ERCP)
Sometimes liver biopsy
The disorder may be suspected when results of liver tests, done as part of an annual physical examination or for some unrelated reason, are abnormal. Then, ultrasound is typically done to check for blockage of bile ducts outside the liver. Tests that can confirm the diagnosis include the following:
Magnetic resonance cholangiopancreatography (MRCP): Magnetic resonance imaging (MRI) is used to obtain images of the bile ducts and the pancreatic duct. This test helps confirm primary sclerosing cholangitis and rule out other causes of bile duct obstruction.
Endoscopic retrograde cholangiopancreatography (ERCP): X-rays are taken after a radiopaque contrast agent, which is visible on x-rays, is injected into the bile ducts through an endoscope (see also figure Understanding Endoscopic Retrograde Cholangiopancreatography). ERCP is less desirable than MRCP because ERCP is more invasive and requires injection of a contrast agent and should be avoided unless required to treat complications of the disorder.
Blood tests and MRCP may be done regularly to check for cancer of the bile ducts and liver tissue.
Liver biopsy is occasionally necessary to determine the specific type of primary sclerosing cholangitis.
When people are diagnosed with primary sclerosing cholangitis, they should also have a colonoscopy with biopsies to determine if they also have inflammatory bowel disease.
Treatment of Primary Sclerosing Cholangitis
Sometimes medications
Endoscopic retrograde cholangiopancreatography (ERCP) to relieve narrowing (strictures)
Sometimes liver transplantation
If people have no symptoms, no treatment is required. But at least twice a year, they need to have a physical examination and blood tests to monitor the progression of the disorder.
The medications ursodeoxycholic acid and cholestyramine may help relieve itching, if it is not relieved by antihistamines. Recurring bacterial cholangitis is treated with antibiotics. Endoscopic retrograde cholangiopancreatography (ERCP) is done as needed to widen (dilate) the blocked ducts. Sometimes tubes to keep the ducts open (stents) are inserted temporarily.The medications ursodeoxycholic acid and cholestyramine may help relieve itching, if it is not relieved by antihistamines. Recurring bacterial cholangitis is treated with antibiotics. Endoscopic retrograde cholangiopancreatography (ERCP) is done as needed to widen (dilate) the blocked ducts. Sometimes tubes to keep the ducts open (stents) are inserted temporarily.
Liver transplantation is the only treatment that prolongs life and can be curative. A liver transplant may be needed in people with cirrhosis and related serious complications, in those with recurrent bacterial cholangitis, or for people with bile duct or liver cancer.
People with primary sclerosing cholangitis should also undergo periodic monitoring for the development of gallbladder, bile duct, and colon cancer, narrowing of the bile ducts, reduced bone density, and vitamin deficiencies.
More Information
The following English-language resources may be useful. Please note that The Manual is not responsible for the content of these resources.
International Foundation for Gastrointestinal Disorders (IFFGD): A resource that helps people with gastrointestinal disorders manage their health.
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK): Comprehensive information on how the digestive system works and links to related topics, such as research and treatment options.
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