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Primary biliary cholangitis

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2025-01

Primary biliary cholangitis is characterised by progressive destruction and disappearance of the intralobular bile duct epithelial cells leading to cholestasis (high alkaline phosphatase and GGT {gamma glutamyl transferase}) and eventually liver cirrhosis and liver failure, generally associated with the presence of circulating antimitochondrial antibodies and an increase of serum IgM levels.

exclusions

• Hepatic fibrosis or cirrhosis  (DB93)
• Alcoholic cirrhosis of liver without hepatitis  (DB94.3)
• Primary sclerosing cholangitis  (DB96.2)

code elsewhere

• Oesophageal varices with bleeding in primary biliary cholangiopathy  (DA26.00)

sections/codes in this section (DB96.1-DB96.1)

• Primary biliary cholangitis with overlap syndrome  (DB96.10)
• Other specified primary biliary cholangitis  (DB96.1Y)
• Primary biliary cholangitis, unspecified  (DB96.1Z)

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