Primary biliary cirrhosis is an inflammation (irritation and swelling with increased presence of immune cells) of the bile ducts of the liver resulting in narrowing and obstruction of the flow of bile . This obstruction damages liver cells.

Alternative Names

PBC

Causes, incidence, and risk factors

The cause of inflamed bile ducts within the liver in this condition is not known. The disease more commonly affects middle-aged women. The onset of symptoms is gradual, most commonly with fatigue and itching skin as the first symptom. Long-standing bile obstruction is believed to lead to liver cirrhosis . The disease may be associated with autoimmune disorders (hypothyroidism, Raynaud's, CREST syndrome, arthridities). There are about 3 to 15 cases per million people in the US population each year.

Signs and tests

Tests that suggest or confirm the disease:

Treatment

Therapy is aimed at symptomatic treatment and prevention of complications. Cholestyramine (or colestipol) may reduce the itching by reducing blood levels of bile acids and increasing clearance of bile acids in the skin. Ursodeoxycholic acid (or actigall) may also improve clearance of bile from the bloodstream. Vitamin A , vitamin K , and vitamin D replacement therapy adds fat-soluble vitamins lost with 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.

Expectations (prognosis)

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.

Complications

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.

Calling your health care provider

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 health care provider if these or other new symptoms develop.