ICD-11 classes
13 Diseases of the digestive system
Diseases of pancreas
DC33 — Autoimmune pancreatitis

ICD-11 DC33 — Autoimmune pancreatitis

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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.