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Primary Sclerosing Cholangitis

By

Christina C. Lindenmeyer

, MD, Cleveland Clinic

Last full review/revision Sep 2021| Content last modified Sep 2021
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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 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 Overview of Gallbladder and Bile Duct Disorders The liver produces bile, a greenish yellow, thick, sticky fluid. Bile aids digestion by making cholesterol, fats, and fat-soluble vitamins easier to absorb from the intestine. Bile also helps... read more Overview of Gallbladder and Bile Duct Disorders .)

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 (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 Primary Biliary Cholangitis (PBC) Primary biliary cholangitis (PBC) is inflammation with progressive scarring of the bile ducts in the liver. Eventually, the ducts are blocked, the liver becomes scarred, and cirrhosis and liver... read more 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 Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers autoimmune disorders is not known. Symptoms vary depending on... read more (when the immune system attacks the body’s own tissues).

Primary sclerosing cholangitis most often affects young men and is diagnosed at an average age of 40. It commonly occurs in people with inflammatory bowel disease Overview of Inflammatory Bowel Disease (IBD) In inflammatory bowel diseases, the intestine (bowel) becomes inflamed, often causing recurring abdominal pain and diarrhea. The two primary types of inflammatory bowel disease (IBD) are Crohn... read more , especially ulcerative colitis Ulcerative Colitis Ulcerative colitis is a chronic inflammatory bowel disease in which the large intestine (colon) becomes inflamed and ulcerated (pitted or eroded), leading to flare-ups (bouts or attacks) of... read more . 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

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 Osteoporosis Osteoporosis is a condition in which a decrease in the density of bones weakens the bones, making breaks (fractures) likely. Aging, estrogen deficiency, low vitamin D or calcium intake, and... read more Osteoporosis , easy bruising and bleeding, and stools that are greasy and foul-smelling (steatorrhea). Gallstones Gallstones Gallstones are collections of solid material (predominantly crystals of cholesterol) in the gallbladder. The liver can secrete too much cholesterol, which is carried with bile to the gallbladder... read more Gallstones and bile duct stones develop in about three fourths of people with primary sclerosing cholangitis. The liver and spleen may enlarge.

Some people have no symptoms until the disorder is advanced and cirrhosis is present. Symptoms may not appear for up to 10 years.

Usually, primary sclerosing cholangitis worsens gradually. Liver failure typically occurs on average approximately 12 years after the disorder is diagnosed.

Diagnosis of Primary Sclerosing Cholangitis

  • Liver blood tests

  • Ultrasonography, followed by other imaging tests

Blood tests and MRCP may be done regularly to check for cancer of the bile ducts and liver tissue.

Treatment of Primary Sclerosing Cholangitis

  • Treatment of symptoms and complications

  • 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 drugs ursodeoxycholic acid and cholestyramine 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.

If cancer of the bile ducts Tumors of the Bile Ducts and Gallbladder Tumors, both noncancerous and cancerous, within the bile ducts or gallbladder are rare. Ultrasonography or MRI/MRCP can usually detect a tumor in the bile ducts or gallbladder. These cancers... read more 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, can improve jaundice, and prevent recurrent infections. If bile duct cancer is limited to the bottom of the liver (where the bile ducts outside of the liver meet the bile ducts inside the liver), a liver transplant Liver Transplantation Liver transplantation is the surgical removal of a healthy liver or sometimes a part of a liver from a living person and then its transfer into a person whose liver no longer functions. (See... read more may be a curative option.

More Information

The following are some English-language resources that may be useful. Please note that THE MANUAL is not responsible for the content of these resources.

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Jaundice is a condition in which the skin and white parts of the eyes appear yellow. It occurs when there is too much bilirubin, a yellow waste product, in the blood. Which of the following is NOT a common cause of jaundice?
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