DC33

ICD-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

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

Sibling codes

  • DC30 – Cystic diseases of the pancreas
  • DC31 – Acute pancreatitis
  • DC32 – Chronic pancreatitis
  • DC34 – Obstructive pancreatitis
  • DC35 – Certain specified diseases of pancreas
  • DC3Y – Other specified diseases of pancreas
  • DC3Z – Diseases of pancreas, unspecified

Foundation entity

Autoimmune pancreatitis – http://id.who.int/icd/entity/2057951941

Allowed postcoordination axes

Part of: 13: Diseases of the digestive system