Clinical and pathophysiological aspects of type 1 autoimmune pancreatitis

J Gastroenterol. 2018 Apr;53(4):475-483. doi: 10.1007/s00535-018-1440-8. Epub 2018 Feb 19.


In 1995, Yoshida and colleagues proposed the concept of "autoimmune pancreatitis" (AIP), which has recently been recognized as a new pancreatic inflammatory disease. Recent studies have suggested the existence of two subtypes of AIP: type 1, which involves immunoglobulin G4 (IgG4) and is the pancreatic manifestation of IgG4-related disease (IgG4-RD); and type 2, which is characterized by granulocytic epithelial lesions. Type 2 AIP is thought to be rare in Japan. Type 1 AIP is characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis. However, although type 1 AIP has become increasingly recognized, many clinical and basic issues remain to be solved. This review provides an overview of the recent clinical and basic knowledge of type 1 AIP.

Keywords: Autoimmune pancreatitis; Basophil; IgG4; M2 macrophage; Regulatory B-cells; Regulatory T-cells.

Publication types

  • Review

MeSH terms

  • B-Lymphocytes, Regulatory / immunology
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunity, Innate
  • Immunoglobulin G4-Related Disease / diagnosis*
  • Immunoglobulin G4-Related Disease / drug therapy
  • Immunoglobulin G4-Related Disease / immunology
  • Immunoglobulin G4-Related Disease / physiopathology
  • Pancreatitis / diagnosis*
  • Pancreatitis / drug therapy
  • Pancreatitis / immunology
  • Pancreatitis / physiopathology
  • Prognosis
  • T-Lymphocytes, Regulatory / immunology


  • Glucocorticoids