Primary biliary cirrhosis

This code identifies a chronic, progressive autoimmune liver disease characterized by the gradual destruction of the small bile ducts within the liver. This destruction leads to cholestasis, inflammation, and eventually cirrhosis and liver failure. It primarily affects middle-aged women.

Assign this code when documentation clearly indicates a diagnosis of primary biliary cirrhosis (PBC). This diagnosis is typically supported by elevated liver enzymes (especially alkaline phosphatase), positive anti-mitochondrial antibodies (AMA), and characteristic liver biopsy findings. Use this code for established diagnoses, not for suspected or ruled-out conditions.

• Differentiate carefully from primary sclerosing cholangitis (K83.01), which is an "Excludes2" condition.
• Ensure documentation specifies "primary" biliary cirrhosis; other forms of biliary cirrhosis may have different codes.
• Consider sequencing with codes for complications such as portal hypertension, ascites, or hepatic encephalopathy, if present.