Extraintestinal Manifestations of Inflammatory Bowel Disease: Autoimmune Pancreatitis and Other IgG4-Related Conditions

  • Terumi Kamisawa
  • Taku Tabata
  • Go Kuwata
  • Koichi Koizumi
Chapter
First Online:

Abstract

Autoimmune pancreatitis (AIP) is a unique pancreatitis with a presumed autoimmune etiology. AIP occurs primarily in elderly males and is characterized clinically by responsiveness to steroid therapy, radiologically by enlargement of the pancreas and irregular narrowing of the main pancreatic duct, serologically by elevation of serum IgG4 levels, and histopathologically by lymphoplasmacytic infiltration and fibrosis in the pancreas (lymphoplasmacytic sclerosing pancreatitis [LPSP]). AIP is currently diagnosed based on a combination of these characteristic features, and it is important to differentiate it from pancreatic cancer. AIP and its various extrapancreatic lesions such as sclerosing cholangitis, sclerosing sialadenitis, and retroperitoneal fibrosis appear to be involved in IgG4-related systemic sclerosing disease. Inflammatory bowel disease (IBD) is rare in LPSP patients but is occasionally associated with another type of AIP called idiopathic duct-centric pancreatitis (IDCP).

Keywords

Autoimmune pancreatitis IgG4 Ulcerative colitis Crohn’s disease 
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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Terumi Kamisawa
    • 1
  • Taku Tabata
    • 1
  • Go Kuwata
    • 1
  • Koichi Koizumi
    • 1
  1. 1.Department of Internal MedicineTokyo Metropolitan Komagome HospitalTokyoJapan

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