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Pathology - Primary biliary cirrhosis
Definition: A chronic liver disease with autoimmune destruction of tiny intrahepatic bile ducts and anti-mitochondrial antibodies.
Epidemiology • Uncommon. • Most common in middle-aged women and linked to other autoimmune disorders. The cause is unknown, but infections with organisms that resemble antigens on the biliary epithelium may be a contributing factor.
Pathogenesis: • Identified as an autoimmune disease when the immune system reacts abnormally to the biliary epithelium. Early stages of the disease may be asymptomatic, but high alkaline phosphatase values may indicate a diagnosis. Patients may experience weariness or pruritus owing to bile salt buildup. More than 95% of cases have anti-mitochondrial antibodies targeting a component of the pyruvate dehydrogenase complex in the inner mitochondrial matrix. Early disease exhibits few macroscopic changes in the liver, while advanced disease causes cirrhosis and bile staining.
Histopathology: • The earliest feature is the invasion and destruction of interlobular bile ducts by lymphocytes and macrophages, known as a 'fluid duct lesion'. Macrophages may form clusters and create granulomas. As the condition advances, it causes inflammation and loss of hepatocytes along the portal tract margins (interface hepatitis), leading to periportal fibrosis, portal-portal bridging, and cirrhosis.
Prognosis: Gradual progression to cirrhosis over 15-20 years. Ursodeoxycholic acid therapy slows the progression.
Definition: A chronic liver disease with autoimmune destruction of tiny intrahepatic bile ducts and anti-mitochondrial antibodies.
Epidemiology • Uncommon. • Most common in middle-aged women and linked to other autoimmune disorders. The cause is unknown, but infections with organisms that resemble antigens on the biliary epithelium may be a contributing factor.
Pathogenesis: • Identified as an autoimmune disease when the immune system reacts abnormally to the biliary epithelium. Early stages of the disease may be asymptomatic, but high alkaline phosphatase values may indicate a diagnosis. Patients may experience weariness or pruritus owing to bile salt buildup. More than 95% of cases have anti-mitochondrial antibodies targeting a component of the pyruvate dehydrogenase complex in the inner mitochondrial matrix. Early disease exhibits few macroscopic changes in the liver, while advanced disease causes cirrhosis and bile staining.
Histopathology: • The earliest feature is the invasion and destruction of interlobular bile ducts by lymphocytes and macrophages, known as a 'fluid duct lesion'. Macrophages may form clusters and create granulomas. As the condition advances, it causes inflammation and loss of hepatocytes along the portal tract margins (interface hepatitis), leading to periportal fibrosis, portal-portal bridging, and cirrhosis.
Prognosis: Gradual progression to cirrhosis over 15-20 years. Ursodeoxycholic acid therapy slows the progression.
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