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A correction has been published: N Engl J Med 2006;354(3):313.

Review Article
Medical Progress
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Volume 353:1261-1273 September 22, 2005 Number 12
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Primary Biliary Cirrhosis
Marshall M. Kaplan, M.D., and M. Eric Gershwin, M.D.

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Primary biliary cirrhosis is a slowly progressive autoimmune disease of the liver that primarily affects women. Its peak incidence occurs in the fifth decade of life, and it is uncommon in persons under 25 years of age. Histopathologically, primary biliary cirrhosis is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. These changes occur at different rates and with varying degrees of severity in different patients. The loss of bile ducts leads to decreased bile secretion and the retention of toxic substances within the liver, resulting in further hepatic damage, fibrosis, cirrhosis, and eventually, liver failure.

Serologically, . . . [Full Text of this Article]

Clinical Findings

Pathological Findings

Natural History and Prognosis

Causes

Epidemiologic and Genetic Factors

Environmental Factors

Autoimmune Responses

Antimitochondrial-Antibody Response

T-Cell Mitochondrial Response

The Bile-Duct Cell and Apoptosis

Antinuclear Antibodies

Treatment of Symptoms and Complications

Pruritus

Osteoporosis

Hyperlipidemia

Portal Hypertension

Treatment of Underlying Disease

Ursodeoxycholic Acid

Colchicine and Methotrexate

Other Drugs

Orthotopic Liver Transplantation

Overview of Treatment

Future Directions


Source Information

From the Division of Gastroenterology, Tufts–New England Medical Center, Boston (M.M.K.); and the Division of Rheumatology, Allergy and Clinical Immunology, School of Medicine, University of California at Davis, Davis (M.E.G.).

Address reprint requests to Dr. Kaplan at the Division of Gastroenterology, Tufts–New England Medical Center, 750 Washington St., Boston, MA 02111, or at mkaplan@tufts-nemc.org.


Related Letters:

Primary Biliary Cirrhosis
Brancatelli G., Federle M. P., Blachar A., Gong Y., Christensen E., Gluud C., Roblin X., Bonaz B., Kaplan M. M., Gershwin M. E.
Extract | Full Text | PDF  
N Engl J Med 2005; 353:2719-2720, Dec 22, 2005. Correspondence

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