DB96.1

ICD-11 MMS 2026-01

Primary biliary cholangitis

Description

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.

Inclusions

  • chronic nonsuppurative destructive cholangitis

Excludes

  • DB93 – Hepatic fibrosis or cirrhosis
  • DB93.Y – Secondary biliary cirrhosis
  • DB94.3 – Alcoholic cirrhosis of liver without hepatitis
  • DB96.2 – Primary sclerosing cholangitis

Exclusions from above levels

  • ME10.1 – Unspecified jaundice
  • 01 – Certain infectious or parasitic diseases
  • 02 – Neoplasms
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  • 05 – Endocrine, nutritional or metabolic diseases
  • 06 – Mental, behavioural or neurodevelopmental disorders
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 22 – Injury, poisoning or certain other consequences of external causes

Child codes

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

Sibling codes

  • DB96.0 – Autoimmune hepatitis
  • DB96.2 – Primary sclerosing cholangitis
  • DB96.Y – Other specified autoimmune liver disease
  • DB96.Z – Autoimmune liver disease, unspecified

Foundation entity

Primary biliary cholangitis – http://id.who.int/icd/entity/649193479

Allowed postcoordination axes

Part of: DB96: Autoimmune liver disease › 13: Diseases of the digestive system