Background and Perspective

  • Daniel S. Longnecker


The recognition and characterization of autoimmune pancreatitis (AIP) and IgG4-related diseases (IgG4-RD) has evolved over the course of more than 50 years. The suggestion that chronic pancreatitis was sometimes caused by autoimmune mechanisms was mentioned in several publications beginning in 1959, but the recognition of characteristic clinical, imaging, and histopathologic features for such patients was not recorded until the 1990s. The characterization of AIP continues to be refined in multiple centers and countries. Heterogeneity in the histopathology among cases delayed the recognition of AIP, but ultimately led to the recognition of two subtypes, one of which occurs in the context of IgG4-RD. The recognition of IgG4-RD can be regarded as an outgrowth of the study of autoimmune pancreatitis.


Chronic Pancreatitis Sclerosing Cholangitis Autoimmune Pancreatitis Alcoholic Pancreatitis Chronic Alcoholic Pancreatitis 
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The author thanks William F. Hickey, Günter Klöppel, and Thomas Smyrk for suggestions during the preparation of this chapter.


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© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of Pathology, Geisel School of Medicine at DartmouthDartmouth-Hitchcock Medical CenterLebanonUSA

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