Section 10. Liver and Gallbladder Disorders

Chapter 136. Fatty Liver, Cirrhosis, and Related Disorders

Topics: Introduction | Fatty Liver | Cirrhosis | Primary Biliary Cirrhosis | Primary Sclerosing Cholangitis | Alpha1-Antitrypsin Deficiency

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis is inflammation and eventual scarring and obstruction of the bile ducts inside and outside the liver.

In primary sclerosing cholangitis, scarring narrows and eventually blocks the bile ducts, leading to cirrhosis. It differs from primary biliary cirrhosis because the bile ducts both inside and outside the liver are affected. The cause is not known but is likely autoimmune, in which the person's immune system attacks the body's own tissues (see Section 16, Chapter 186). The disorder most often affects young men. It commonly occurs in people with inflammatory bowel disease, especially ulcerative colitis.

Symptoms and Complications

Symptoms usually begin gradually with worsening fatigue, itchiness, and jaundice (yellowish discoloration of the skin and the whites of the eyes). Attacks of upper abdominal pain and fever caused by inflammation and recurring infection of the bile ducts (bacterial cholangitis) may occur. However, bacterial cholangitis is uncommon unless the duct system has been manipulated. Sometimes stents, which keep the bile ducts open, are placed endoscopically, and this procedure can result in bacterial cholangitis. An affected person may have an enlarged liver and spleen or symptoms of cirrhosis (see Section 10, Chapter 136). The person also may develop 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), and liver failure, which can be fatal.

Cancer of the bile ducts (cholangiocarcinoma) develops in 10 to 15% of people with primary sclerosing cholangitis.

Diagnosis

Because some people have no symptoms for as long as 10 years, the disorder may be detected by abnormal results of liver function tests performed as part of an annual physical examination or for some unrelated medical reason. The diagnosis is usually confirmed by endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous cholangiography (see Section 10, Chapter 134). In ERCP, x-rays are taken after a radiopaque dye, which is visible on x-rays, is injected into the bile ducts through an endoscope (a flexible viewing tube). In percutaneous cholangiography, x-rays are taken after a radiopaque dye is injected directly into the bile ducts. Increasingly, abdominal ultrasound and especially magnetic resonance imaging (MRI) scans of the bile ducts (magnetic resonance cholangiography) can be used to make the diagnosis. A liver biopsy (removal of a tissue sample for examination under a microscope (see Section 10, Chapter 134)) may be necessary to help confirm the diagnosis.

Prognosis and Treatment

Usually, primary sclerosing cholangitis worsens gradually. Drugs such as corticosteroids, azathioprine, penicillamine, and methotrexate have been used in an attempt to slow the progression of the disorder but have not proved very effective and can cause severe side effects. The value of the drug ursodeoxycholic acid remains unclear. Primary sclerosing cholangitis may require liver transplantation (see Section 16, Chapter 187), which is the only known cure for this otherwise fatal disorder.

Recurring infection of the bile ducts (bacterial cholangitis) requires treatment with antibiotics and, when possible, drainage of obstructed parts of the bile ducts using ERCP.

If cancer of the bile ducts (cholangiocarcinoma) develops and removal of the cancer is not possible by surgery, an endoscope may be used to place tubes (stents) into major bile ducts obstructed by the cancer, to open them up.