Autoimmune pancreatitis

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  • IgG4-related autoimmune process

Diagnostic Criteria (HISORt)

  • Histology suggesting autoimmune pancreatitis
  • Imaging suggestive of autoimmune pancreatitis
  • Serology showing IgG4 ≥2x ULN
  • Other organ involvement: biliary strictures, parotid/lacrimal gland involvement, mediastinal lymphadenopathy, retroperitoneal fibrosis
  • Response to treatment with steroids

Management

  • Prednisone 40 mg/day for four to six weeks then taper by 5 mg weekly
    • Typically requires 10 mg/day for maintenance
  • Repeat imaging to assess for improvement