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Autoimmune pancreatitis type 1

Disease definition

Type 1 autoimmune pancreatitis is a form of autoimmune pancreatitis seen in elderly males (>60 years) and presenting with abdominal pain, steatorrhea, obstructive jaundice and other organ (bile duct, kidneys and retroperitoneum) involvement. It is thought to be due to an immunoglobulin G4 (IgG4)-associated systemic disease.

ORPHA:280302

Classification level: Subtype of disorder
  • Synonym(s):
    • AIP type 1
    • IgG4-related pancreatitis
    • Lymphoplasmacytic sclerosing pancreatitis
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: K86.1
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

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