Primary biliary cirrhosis overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Primary biliary cirrhosis is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts (bile canaliculi) within the liver. When these ducts are damaged bile builds up in the liver (cholestasis) and over time damages the tissue. This can lead to scarring, fibrosis, cirrhosis, and ultimately liver failure.
Historical Perspective
Addison and Gull in 1851 described the clinical picture of progressive obstructive jaundice in the absence of mechanical obstruction of the large bile ducts. Ahrens et al in 1950 coined the term primary biliary cirrhosis for this disease. The association with anti mitochondrial antibodies was first reported in 1986.[1]
References
- ↑ Mitchison HC, Bassendine MF, Hendrick A; et al. (1986). "Positive antimitochondrial antibody but normal alkaline phosphatase: is this primary biliary cirrhosis?". Hepatology. 6 (6): 1279–84. doi:10.1002/hep.1840060609. PMID 3793004.