Abstract
Autoimmune pancreatitis is a benign process characterized by inflammation and fibrosis.
It is now known that cases of “autoimmune pancreatitis” actually consist of two distinct
pathologic entities. Type 1 autoimmune pancreatitis is a manifestation of a systemic
process, immunoglobulin G subclass 4 (IgG4)-related disease. IgG4-related disease
can affect virtually every organ system in the body. Type 1 affects older patients
and is characterized by an elevated serum IgG4 level and sites of extrapancreatic
disease. Type 2 autoimmune pancreatitis is a disease process confined to the pancreas.
It affects younger patients and is associated with inflammatory bowel disease. Type
2 is not associated with elevated IgG4 levels or extrapancreatic disease. Both subtypes
can mimic malignancy, particularly pancreatic cancer. Awareness of the clinical and
imaging features of the subtypes of autoimmune pancreatitis is important to avoid
an incorrect diagnosis of malignancy.
Keywords
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Article info
Publication history
Published online: May 14, 2014
Footnotes
Funding: None.
Conflict of Interest: None.
Authorship: All authors had access to the data and played a role in writing this manuscript.
Identification
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© 2014 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
