RECENT ADVANCES IN BASIC SCIENCE
Pathogenesis of primary biliary cirrhosis
Liver Research Group, Institute of Cellular Medicine, University of Newcastle, Newcastle-upon-Tyne, UK
Correspondence to:
Professor David E J Jones, Liver Research Group, Institute of Cellular Medicine, 4th Floor William Leech Building, The Medical School, Framlington Place, Newcastle-upon-Tyne NE2 4HH, UK; d.e.j.jones@ncl.ac.uk
| The first 150 words of the full text of this article appear below. |
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that affects up to one in 700 women in western populations (female to male ratio is approximately 10 to 1, with the disease typically presenting over the age of 40 years).1 2 PBC is characterised histologically by damage to, and eventual loss of, the biliary epithelial cells (BEC) lining small intrahepatic bile ducts. BEC loss is typically accompanied by a significant portal tract inflammatory infiltrate that is mixed in phenotype (T cells (CD4 and CD8, with the latter predominating in the periductal areas), B cells, macrophages, eosinophils and natural killer cells).3 4 Early descriptions of PBC emphasised the predominant role played by the progression of bile duct loss, accompanied by increasing portal tract and linking fibrosis leading to biliary cirrhosis, in the clinical expression of PBC, and described an aggressive and uniformly fatal condition.5 Increased awareness of the condition and, in particular,
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