Primary biliary cirrhosis
Key points
- Primary biliary Cirrhosis is an inflammation (irritation and swelling) of the bile ducts of the liver resulting in narrowing and obstruction of the flow of bile.
- The cause of inflamed bile ducts within the liver in this condition is not known.
- Therapy is aimed at relief of symptoms and prevention of complications.
- The course is variable, but if the condition is untreated, most patients will need a liver transplant after about seven years.
Alternative names
PBC
Definition
Primary biliary Cirrhosis is an inflammation (irritation and swelling) of the bile ducts of the liver resulting in narrowing and obstruction of the flow of bile. This obstruction damages liver cells.
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, with fatigue and itching skin as the most common first symptom.
Long-standing bile obstruction is believed to lead to liver cirrhosis. The disease may be associated with autoimmune disorders such as Hypothyroidism, Raynaud's, CREST syndrome, and arthridities. There are about 3 to 15 cases per million people in the U.S. population each year.
Symptoms
- Itching
- Jaundice
- Enlarged liver
- Abdominal pain
- Fatty deposits under the skin
- Soft yellow spots on the eyelid
- Fatty stools
Signs and tests
Tests that suggest or confirm the disease:
- Liver biopsy
- Mitochondrial antibodies (positive in about 95% of cases)
Tests that reveal liver dysfunction:
- Liver function tests
- Serum cholesterol and lipoproteins may be increased
Treatment
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 clearance of bile acids in the skin. Ursodeoxycholic acid may also improve clearance 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.
Written and revised by
Gerald Hurowitz, M.D.
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