Primary biliary cirrhosis: From induction to destruction

A. Nishio, N. M. Bass, V. A C Luketic, R. L. Coppel, M. Eric Gershwin

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that predominantly affects middle-aged women; fatigue and pruritus are the most common symptoms at presentation. Liver function tests are consistent with cholestasis and reveal an elevation of serum alkaline phosphatase and γ-glutamyl transpeptidase with or without elevation of aminotransferase levels. Histologically, PBC is characterized by the destruction of the intrahepatic small bile ducts and subsequently fibrosis. The serological hallmark of the disease is the presence of antimitochondrial antibodies, which are found in 95% of patients with PBC. The antimitochondrial antibodies are directed against the 2-oxo-acid dehydrogenase complexes located on the inner membrane of the mitochondria. PBC generally slowly progresses, even over decades, and may lead to liver failure. In symptomatic patients, advanced age, elevated serum bilirubin levels, decreased serum albumin levels, and cirrhosis each correlate with shortened survival. Immunosuppressive and anti-inflammatory drugs have been used in the treatment of PBC based on the presumed autoimmune pathogenesis, but satisfactory agents leading to complete reversal or cure of the disease are not available. At present ursodeoxycholic acid appears to be the only effective therapy in preventing or delaying the need for liver transplantation and improving survival. However, a number of patients receiving ursodeoxycholic acid still develop progressive disease and require transplantation; transplantation is the only effective therapy at the end stage of the disease.

Original languageEnglish (US)
Pages (from-to)89-102
Number of pages14
JournalSeminars in Gastrointestinal Disease
Volume12
Issue number2
StatePublished - 2001

Fingerprint

Biliary Liver Cirrhosis
Ursodeoxycholic Acid
Fibrosis
Transplantation
Keto Acids
Intrahepatic Bile Ducts
Survival
gamma-Glutamyltransferase
Antibodies
Liver Function Tests
Cholestasis
Liver Failure
Pruritus
Immunosuppressive Agents
Transaminases
Serum
Bilirubin
Serum Albumin
Liver Transplantation
Fatigue

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Nishio, A., Bass, N. M., Luketic, V. A. C., Coppel, R. L., & Gershwin, M. E. (2001). Primary biliary cirrhosis: From induction to destruction. Seminars in Gastrointestinal Disease, 12(2), 89-102.

Primary biliary cirrhosis : From induction to destruction. / Nishio, A.; Bass, N. M.; Luketic, V. A C; Coppel, R. L.; Gershwin, M. Eric.

In: Seminars in Gastrointestinal Disease, Vol. 12, No. 2, 2001, p. 89-102.

Research output: Contribution to journalArticle

Nishio, A, Bass, NM, Luketic, VAC, Coppel, RL & Gershwin, ME 2001, 'Primary biliary cirrhosis: From induction to destruction', Seminars in Gastrointestinal Disease, vol. 12, no. 2, pp. 89-102.
Nishio, A. ; Bass, N. M. ; Luketic, V. A C ; Coppel, R. L. ; Gershwin, M. Eric. / Primary biliary cirrhosis : From induction to destruction. In: Seminars in Gastrointestinal Disease. 2001 ; Vol. 12, No. 2. pp. 89-102.
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