Overview
The Disease Process
Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic autoimmune disease in which the small bile ducts within the liver are progressively da...
Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic autoimmune disease in which the small bile ducts within the liver are progressively damaged. Bile can no longer flow normally from hepatocytes into the biliary system. The resulting cholestasis causes bile constituents to accumulate in the liver, where ongoing injury stimulates inflammation and fibrosis. This is not the same process as acute ascending cholangitis. PBC is usually gradual and immune-mediated rather than an acute bacterial infection caused by an obstructed common bile duct. Fever, rigors, acute right-upper-quadrant pain, or haemodynamic instability should therefore prompt assessment for another urgent hepatobiliary problem rather than being attributed automatically to PBC. Early disease may be found incidentally when alkaline phosphatase (ALP) is abnormal. As cholestasis progresses, patients may report fatigue or pruritus before jaundice appears. Later disease can impair hepatic synthetic function and progress to cirrhosis, portal hypertension, and liver failure. The nurse’s clinical reasoning follows that trajectory: identify a cholestatic pattern, determine whether the liver is still compensated, and detect a change that requires urgent escalation.
