Doctor's Notes on Primary Biliary Cholangitis and Cirrhosis (PBC)
Primary biliary cirrhosis, also referred to as primary biliary cholangitis, is a condition in which there is progressive damage to the small bile ducts within the liver. This results in a backup of bile in the liver. The pressure build-up further damages the bile ducts asnd causes damage to liver cells. Over time, the disease progresses and liver cells begin to die, resulting in cirrhosis (scarring of the liver) and liver failure. The cause of primary biliary cirrhosis is not well understood. It is thought to be an autoimmune disease, in which the body’s immune system attacks the cells of the bile ducts.
Symptoms of primary biliary cirrhosis include fatigue and itching skin, although up to 25% of patients with the condition do not have any symptoms when it is first discovered through abnormalities in blood tests. Associated symptoms can include dryness of the mouth and eyes. Inflammation of the liver can cause abdominal pain. If scarring (cirrhosis) is present, symptoms can include fluid accumulation in the abdomen (ascites), jaundice, swelling of the legs, and muscle wasting.
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Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.