Autoimmune pancreatitis and IgG4-related systemic diseases

Lizhi Zhang, Thomas C. Smyrk

Research output: Contribution to journalReview articlepeer-review

72 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)491-504
Number of pages14
JournalInternational Journal of Clinical and Experimental Pathology
Issue number5
StatePublished - 2010


  • Autoimmune pancreatitis
  • IgG4
  • IgG4-related systemic disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology


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