Primary biliary cirrhosis

• A
• B
• C
• D
• E
• F
• G
• H
• I
• J
• K
• L
• M
• N
• O
• P
• Q
• R
• S
• T
• U
• V
• W
• X
• Y
• Z

Bile pathway

Digestive system

PBC

Therapy is aimed at relief of symptoms and prevention of complications.

Cholestyramine (or colestipol) may reduce the itching by reducing blood levels of bile acids and increasing removal of bile acids in the skin. Ursodeoxycholic acid may also improve removal of bile from the bloodstream.

Vitamin replacement therapy restores fat-soluble vitamins A, K, and D lost in fatty stools. A calcium supplement is indicated to prevent osteomalacia.

Periodic evaluation and drainage with ERCP  (endoscopic retrograde cholangiopancreatography) or PTC (percutaneous transhepatic cholangiography) may be necessary.

Treatment for episodes of cholangitis may be necessary.

Liver transplant for primary biliary cirrhosis prior to liver failure has been successful.

The course is variable, but if the condition is untreated, most patients will need a liver transplant after about seven years. Statistical models are now used to predict the best timing for transplantation.

Progressive cirrhosis can lead to liver failure -- including bleeding, malnutrition, renal failure, fluid, and electrolyte imbalance -- and encephalopathy (damage to the brain). Osteodystrophy (a condition that softens the bones) and malabsorption may develop.

Call your health care provider if prolonged itching of the skin develops that is not related to other causes, or if jaundice or other symptoms of cirrhosis develop.

Vomiting blood , blood in the stools , confusion, abdominal swelling, and jaundice are symptoms that indicate cirrhosis may be worsening. Call your provider if these or other new symptoms develop.

Review Date: 05/04/2006
Reviewed By: Jenifer K. Lehrer, MD, Department of Gastroenterology, Frankford-Torresdale Hospital, Jefferson Health System, Philadelphia, PA. Review provided by VeriMed Healthcare Network.