Primary biliary cirrhosis
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. It was previously thought to be a rare disease, but more recent studies have shown that it may affect up to 1 in 3-4,000 people; the sex ratio is not less than 9 to 1, women to men (reference: Medical care of the Liver Trasplant Patient, 3rd Edition published 2006, editied by Paul G. Killenberg, page 155).
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Primary biliary cirrhosis
Primary biliary cirrhosis (PBC)
A liver disease caused by an abnormality of the immune system. Small bile ducts within the liver become inflamed and obliterated. Backup of bile causes intense skin itching and yellowing of the skin (jaundice). Lack of bile decreases absorption of calcium and vitamin D, leading to osteoporosis. Cirrhosis (scarring of the liver) develops over time.