Recent Advances in Autoimmune Pancreatitis

Phil A. Hart, Yoh Zen, Suresh T Chari

Research output: Contribution to journalArticle

100 Citations (Scopus)

Abstract

Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is characterized clinically by frequent presentation with obstructive jaundice, histologically by a dense lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to corticosteroid therapy. Two distinct diseases, type 1 and type 2 AIP, share these features. However, these 2 diseases have unique pancreatic histopathologic patterns and differ significantly in their demographic profiles, clinical presentation, and natural history. Recognizing the popular and long-standing association of the term "AIP" with what is now called "type 1 AIP," we suggest using "AIP" solely for type 1 AIP and to acknowledge its own distinct disease status by using "idiopathic duct-centric chronic pancreatitis" (IDCP) for type 2 AIP. AIP is the pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD). The etiopathogenesis of AIP and IgG4-RD is largely unknown. However, the remarkable effectiveness of B-cell depletion therapy with rituximab in patients with AIP and IgG4-RD highlights the crucial role of B cells in its pathogenesis. IDCP is less commonly recognized, and little is known about its pathogenesis. IDCP has no biomarker but is associated with inflammatory bowel disease in ∼25% of patients. Recently, the international consensus diagnostic criteria for AIP identified combinations of features that are diagnostic of both diseases. Both AIP and IDCP are corticosteroid responsive; however, relapses are common in AIP and rare in IDCP. Therefore, maintenance therapy with either an immunomodulator (eg, azathioprine, 6-mercaptopurine, or mycophenolate mofetil) or rituximab is often necessary for patients with AIP. Long-term survival is excellent for both patients with AIP and patients with IDCP.

Original languageEnglish (US)
Article number59669
Pages (from-to)39-51
Number of pages13
JournalGastroenterology
Volume149
Issue number1
DOIs
StatePublished - Jul 1 2015

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Pancreatitis
Chronic Pancreatitis
Immunoglobulins
Adrenal Cortex Hormones
B-Lymphocytes
Mycophenolic Acid
6-Mercaptopurine
Obstructive Jaundice
Azathioprine
Immunologic Factors
Cell- and Tissue-Based Therapy
Natural History
Inflammatory Bowel Diseases
Fibrosis
Biomarkers
Demography

Keywords

  • Idiopathic Duct-Centric Chronic Pancreatitis
  • IgG4-Related Disease
  • Lymphoplasmacytic Sclerosing Pancreatitis
  • Rituximab

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Recent Advances in Autoimmune Pancreatitis. / Hart, Phil A.; Zen, Yoh; Chari, Suresh T.

In: Gastroenterology, Vol. 149, No. 1, 59669, 01.07.2015, p. 39-51.

Research output: Contribution to journalArticle

Hart, Phil A. ; Zen, Yoh ; Chari, Suresh T. / Recent Advances in Autoimmune Pancreatitis. In: Gastroenterology. 2015 ; Vol. 149, No. 1. pp. 39-51.
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