Sclerosing cholangitis

Definition

Sclerosing cholangitis refers to swelling (inflammation), scarring, and destruction of the bile ducts inside and outside of the liver.

Causes, incidence, and risk factors

In this condition, the bile ducts inside and outside the liver become narrowed and scarred.

The cause is often unknown.

The disease is often seen in patients who have:

• Inflammatory bowel disease (Crohn's disease and ulcerative colitis)
• Sarcoidosis
• Chronic pancreatitis
• Many autoimmune disorders

Genetic factors may also be responsible. Sclerosing cholangitis occurs more often in men than women. This disorder is rare in children.

Sclerosing cholangitis may also be caused by:

• Choledocholithiasis
• Toxicity from chemicals, copper, bile acids
• Infections in the liver, gallbladder, and bile ducts

Symptoms

The first symptoms are usually fatigue, yellowing of the skin and eyes (jaundice), and itching. However, some people may have no symptoms.

Other symptoms may include:

• Loss of appetite and weight loss
• Enlarged liver
• Enlarged spleen
• Repeat episodes of cholangitis

Signs and tests

Some people do not have symptoms, but blood work reveals they have abnormal liver function. The doctor will rule out diseases that cause similar problems. For example, you will be checked for stones in the gallbladder or biliary tract.

Tests that show cholangitis include:

• Abdominal CT scan
• Abdominal ultrasound
• Endoscopic retrograde cholangiopancreatography (ERCP)
• Liver biopsy
• Magnetic resonance cholangiopancreatography (MRCP)
• Percutaneous transhepatic cholangiogram (PTC)

Blood tests include:

• Elevated liver enzymes (liver function tests)

Treatment

• Cholestyramine
• Ursodeoxycholic acid (ursodiol)
• Fat-soluble vitamins (D, E, A, K)
• Antibiotics for infections in the bile ducts
• Medications that quiet the immune system (prednisone, azathioprine, cyclosporine, methotrexate)

Surgical procedures:

• Inserting a long, thin tube with a balloon at the end to open up narrowing (endoscopic balloon dilation of strictures)
• Placement of a drain or tube for major narrowing (strictures) of biliary ducts
• Proctocolectomy (for those who have both ulcerative colitis and sclerosing cholangitis)
• Liver transplant

Expectations (prognosis)

How well patients do varies. The disease tends to get worse over time and most patients develop:

• Biliary cirrhosis
• Liver failure
• Persistent jaundice

Some patients develop infections of the bile ducts that keep returning. Patients may have chronic liver disease that gets worse, with ascites and varices.

People with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma). They should be checked on a regular basis (usually each year) with liver scans and blood tests.

Complications

• Bleeding esophageal varices
• Cancer in the bile ducts (cholangiocarcinoma)
• Cirrhosis and liver failure
• Infection of the biliary system (cholangitis)
• Narrowing of the bile ducts (strictures)
• Vitamin deficiencies

References

Gordon FD. Primary sclerosing cholangitis. Surg Clin North Am. 2008;88:1385-1407.

Tung BY, Kowdley KV. Sclerosing cholangitis and recurrent pyogenic cholangitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier;2006:chap 65.