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Int J Clin Exp Pathol 2010;3(5):491-504

Review Article
Autoimmune pancreatitis and IgG4-related systemic diseases

Lizhi Zhang, Thomas C Smyrk

Division of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota.

Received May 5, 2010, accepted May 18, 2010, available online May 25, 2010

Abstract: Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis that is characterized by lymphoplasmacytic infiltrate, storiform
fibrosis, obliterative phlebitis, and increased IgG4+ plasma cells. Serum IgG4 levels usually are elevated. Patients with AIP frequently have
disease affecting other organs or sites; these tissues show similar histologic changes, including increased IgG4+ plasma cell infiltrate and
response to corticosteroid therapy. A new clinicopathologic concept of IgG4-related systemic disease (ISD) has been proposed. These
diseases often are not limited to the pancreas, and the pancreas may not be involved at all. In this article, we review the literature and our own
experience to detail the clinicopathologic features of AIP and extrapancreatic lesions in ISD. (IJCEP1005001).

Key words: autoimmune pancreatitis; IgG4; IgG4-related systemic disease

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Address all correspondence to:
Lizhi Zhang, MD
Division of Pathology
Mayo Clinic
200 First St SW
Rochester, MN 55905
USA.
E-mail:
zhang.lizhi@mayo.edu