Autoimmune pancreatitis pathophysiology

Autoimmune pancreatitis Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Genetics

• [Disease name] is transmitted in [mode of genetic transmission] pattern.
• Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
• The development of [disease name] is the result of multiple genetic mutations.

Associated Conditions

• Autoimmune pancreatitis may be associated with systemic autoimmune conditions such as:
  • IgG4-associated cholangitis
  • Chronic sclerosing sialadenitis (Küttner's tumor)
  • Mikulicz's disease (IgG4-related plasmacytic exocrinopathy)
  • Mediastinal fibrosis, adenopathy
  • Chronic periaortitis
  • Idiopathic retroperitoneal fibrosis
  • Tubulointerstitial nephritis
  • IgG4-associated pseudolymphoma
  • Ulcerative colitis

Gross Pathology

• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

• Microscopic findings suggestive of autoimmune pancreatitis may include:

Type 1 AIP:

• A lymphoplasmacytic sclerosing pancreatitis
• >10 IgG4-positive cells with at least two of the following
  • Periductal lymphoplasmacytic infiltrate
  • Obliterative phlebitis
  • Acinar fibrosis

Type 2 AIP or Idiopathic duct-centric pancreatitis (IDCP):

• Pancreatic duct: Granulocytic epithelial lesion
• Pancreatic parenchyma: Minimal IgG4-positive cells