Autoimmune Pancreatitis

Shounak Majumder, Naoki Takahashi, Suresh T Chari

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Autoimmune pancreatitis (AIP) is a chronic fibroinflammatory disease of the pancreas that belongs to the spectrum of immunoglobulin G-subclass4-related diseases (IgG4-RD) and typically presents with obstructive jaundice. Idiopathic duct-centric pancreatitis (IDCP) is a closely related but distinct disease that mimics AIP radiologically but manifests clinically most commonly as recurrent acute pancreatitis in young individuals with concurrent inflammatory bowel disease. IgG4 levels are often elevated in AIP and normal in IDCP. Histologically, lymphoplasmacytic acinar inflammation and storiform fibrosis are seen in both. In addition, the histologic hallmark of IDCP is the granulocyte epithelial lesion: intraluminal and intraepithelial neutrophils in medium-sized and small ducts with or without granulocytic acinar inflammation often associated with destruction of ductal architecture. Initial treatment of both AIP and IDCP is with oral corticosteroids for duration of 4 weeks followed by a gradual taper. Relapses are common in AIP and relatively uncommon in IDCP, a relatively rare disease for which the natural history is not well understood. For patients with relapsing AIP, treatment with immunomodulators and more recently rituximab has been recommended. Although rare instances of pancreaticobiliary malignancy has been reported in patients with AIP, overall the lifetime risk of developing pancreatic cancer does not appear to be elevated.

Original languageEnglish (US)
Pages (from-to)1-8
Number of pages8
JournalDigestive Diseases and Sciences
DOIs
StateAccepted/In press - Apr 1 2017

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Pancreatitis
Immunoglobulin G
Inflammation
Obstructive Jaundice
Immunologic Factors
Rare Diseases
Natural History
Pancreatic Neoplasms
Inflammatory Bowel Diseases
Granulocytes
Autoimmune Diseases
Pancreas
Adrenal Cortex Hormones
Neutrophils
Fibrosis
Chronic Disease
Recurrence

Keywords

  • Autoimmune pancreatitis
  • Corticosteroids
  • Idiopathic duct-centric pancreatitis
  • Immunomodulator
  • Medical management
  • Relapse
  • Rituximab

ASJC Scopus subject areas

  • Physiology
  • Gastroenterology

Cite this

Autoimmune Pancreatitis. / Majumder, Shounak; Takahashi, Naoki; Chari, Suresh T.

In: Digestive Diseases and Sciences, 01.04.2017, p. 1-8.

Research output: Contribution to journalArticle

Majumder, Shounak ; Takahashi, Naoki ; Chari, Suresh T. / Autoimmune Pancreatitis. In: Digestive Diseases and Sciences. 2017 ; pp. 1-8.
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