Clinical and pathophysiological aspects of type 1 autoimmune pancreatitis

JOURNAL OF GASTROENTEROLOGY

Fecha de publicación: 19 February 2018

DOI: https://doi.org/10.1007/s00535-018-1440-8

Autores: Kazushige Uchida, Kazuichi Okazaki

Background: 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.

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