DC33ICD-11 MMS 2026-01
Autoimmune pancreatitis
Description
Autoimmune pancreatitis (AIP) is a rare pancreatic disease characterised by chronic non-alcoholic pancreatitis that presents with abdominal pain, steatorrhoea, obstructive jaundice and responds well to steroid therapy and is seen in two subforms: type 1 AIP which affects elderly males, involves other organs and has increased immunoglobin G4 (IgG4) levels and type 2 AIP which affects both sexes equally but presents at a younger age and has no other organ involvement or increased IgG4 levels.
Index terms
- Autoimmune pancreatitis
- Type 1 IgG4 related autoimmune pancreatitis
- LPSP - [lymphoplasmacytic sclerosing pancreatitis]
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- Autoimmune pancreatitis with other organ involvement
- Autoimmune pancreatitis without other organ involvement
- Type 2 Autoimmune pancreatitis featuring granulocytic epithelial lesion
- IDCP - [idiopathic duct-centric chronic pancreatitis]
- Type 2 Autoimmune pancreatitis featuring GEL
Exclusions from above levels
Sibling codes
Foundation entity
Autoimmune pancreatitis – http://id.who.int/icd/entity/2057951941
Allowed postcoordination axes
Part of: 13: Diseases of the digestive system