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.)
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 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 most often affects young men and is diagnosed 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.
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 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 typically occurs on average approximately 12 years after the disorder is diagnosed.
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, ultrasonography is typically done first 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 Figure: Understanding Endoscopic Retrograde Cholangiopancreatography). ERCP is less desirable than MRCP because ERCP is more invasive and requires injection of a contrast agent. However, ERCP can also sometimes be used 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.
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 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.
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, 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 may be a curative option.