Article Sections

Introduction

Primary biliary cholangitis (PBC), formerly primary biliary cirrhosis, is a chronic autoimmune liver disease characterized by progressive destruction of intrahepatic bile ducts, leading to cholestasis, liver fibrosis, and eventually cirrhosis.  It predominantly affects middle-aged women.

Pathophysiology and pathology

PBC is characterized by T-cell–mediated destruction of the small intralobular bile ducts within the liver, leading to chronic ductal inflammation and obliteration ( Figure 1).  Histologically, PBC is characterized by a dense lymphocytic infiltrate along the portal tracts with granulomas that obliterate the interlobular bile ducts (ie, florid duct lesions) ( Image 1).  Persistent inflammation and ductal destruction result in hepatic fibrosis and ultimately cirrhosis.

The hallmark autoantibodies seen in PBC are anti-mitochondrial antibodies (AMA), which target the M2 subtype of mitochondrial autoantigens.  AMA has a high sensitivity (~95%) and specificity (~95%) for PBC.

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Figures

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Figure 1

Images

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Tables

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