Digestive Diseases and Sciences

, Volume 62, Issue 7, pp 1762–1769 | Cite as

Autoimmune Pancreatitis

  • Shounak Majumder
  • Naoki Takahashi
  • Suresh T. ChariEmail author


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.


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


Compliance with ethical standards

Conflicts of interest

None of the authors have any conflicts of interest to disclose.


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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Shounak Majumder
    • 1
  • Naoki Takahashi
    • 2
  • Suresh T. Chari
    • 1
    Email author
  1. 1.Division of Gastroenterology and HepatologyMayo ClinicRochesterUSA
  2. 2.Division of RadiologyMayo ClinicRochesterUSA

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