Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a disease that inflames, scars, and blocks bile ducts inside and outside the liver. The bile ducts are small tubes that carry bile out of the liver to the gallbladder and the small intestine. Bile is a liquid formed by the liver, which when release in the small helps break down fat in food.

In PSC, the bile ducts become inflamed, which can lead to scar formation and narrowing of the ducts over time. Continuous scarring of the ducts can cause them to become totally blocked. Total blockage of the bile ducts cause build up bile in the liver, which can damage liver cells. The scar tissue can spread throughout the liver, causing cirrhosis and liver failure.

PSC occurs more often in adults but the disease may also affect children. Risk of developing the disease increases when a family member has PSC. The condition is more common in men than in women and the average age at diagnosis is 40.

Causes

The cause of primary sclerosing cholangitis is not fully known. However, experts believe that genes, problems with the immune system, bacteria, and viruses may play roles in the development of the disease.

Also, PSC is linked to inflammatory bowel disease (IBD). The link between PSC and IBD, however, is not fully inderstood. About three out of four people with PSC have ulcerative colitis—a type of IBD.

Symptoms

PSC develops and progresses slowly, making a person with the disease symptoms-free for many years. When symptoms do appear, the person may experience itching, fatigue, and yellowing of the skin and whites of the eyes. Fever and chills may occur when the bile ducts are infected.

Signs and symptoms of PSC may resemble signs of other conditions or health problems, a consultation with your doctor is necessary.

Complications:

Various complications may result from PSC, including

 deficiencies of vitamins A, D, E, and K

 infections of the bile ducts

 cirrhosis—extensive scarring of the liver

 liver failure

 bile duct cancer

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Image Credit: National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

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Primary Sclerosing Cholangitis cont'd

Diagnosis

In addition to learning about your signs and symptoms, medical and family history, and performing physical examination, your doctor will also test a sample of your blood. Blood tests can be used to check for levels of liver enzymes. Cholangiography, which provides images of the bile ducts, is used by the doctor to confirm the diagnosis.

Cholangiography can be performed in the following ways:

• Endoscopic retrograde cholangiopancreatography (ERCP). ERCP uses an endoscope—a long, flexible, lighted tube—that goes down the mouth, beyond the stomach, and into the duodenum to reach an area in the digestive tract where dye can be injected into the bile ducts. X rays are taken when the dye is injected. ERCP also can be used to take a tissue sample or to treat blocked ducts.


• Percutaneous transhepatic cholangiography. This procedure involves inserting a needle through the skin and placing a thin tube into a duct in the liver. Dye is injected through the tube and x rays are taken.


• Magnetic resonance cholangiopancreatography (MRCP). MRCP uses magnetic resonance imaging (MRI) to obtain pictures of the bile ducts. MRI machines use radio waves and magnets to scan internal organs and tissues. MRCP does not involve using x rays or inserting instruments into the body. This safe and painless test is increasingly used for diagnosis.

The doctor may also order additional tests such as ultrasound exams and a liver biopsy.

Treatment

There is no standard procedure to slow down or stop the progression of PSC. The aim of treatment is to relieve symptoms and manage complications. Medical treatment may include:

 medications to relieve itching

 antibiotics to treat infections

 vitamin supplements

Instruments passed through an endoscope during ERCP can help open blocked bile ducts.

When the liver begins to fail, liver transplantation may be an option.

Source: Adapted from the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Web URL: http://www.niddk.nih.gov

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