ICD-11 classes
04 Diseases of the immune system
Organ specific autoimmune disorders
DB96 — Autoimmune liver disease
DB96.1 — Primary biliary cholangitis
ICD-11 DB96.1 — Primary biliary cholangitis
Primary biliary cholangitis is characterised by progressive destruction and disappearance of the intralobularbile 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.
It includes 1 item.
- chronic nonsuppurative destructive cholangitis
It excludes 3 items.
- Hepatic fibrosis or cirrhosis (DB93)
- Alcoholic cirrhosis of liver without hepatitis (DB94.3)
- Primary sclerosing cholangitis (DB96.2)
Diagnosis with code DB96.1 contains 3 clarifying diagnoses:
- DB96.10 — Primary biliary cholangitis with overlap syndrome
- DB96.1Y — Other specified primary biliary cholangitis
- DB96.1Z — Primary biliary cholangitis, unspecified
The diagnosis is coded elsewhere:
- Autoimmune liver disease #14319
- Primary biliary cholangitis #14321