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Autoimmune Pancreatitis and Other Organ Involvement

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Crohn's Disease and Ulcerative Colitis

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Abstract

Autoimmune pancreatitis (AIP) is a peculiar form of pancreatitis that should be differentiated from pancreatic cancer. AIP is divided into two subtypes: type 1 and type 2. The histologic characteristic of type 1 AIP is lymphoplasmacytic sclerosing pancreatitis (LPSP) showing abundant infiltration of lymphocytes and IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis, while that of type 2 AIP is idiopathic duct-centric chronic pancreatitis (IDCP) showing granulocytic infiltration of the pancreatic duct wall (GEL). AIP is diagnosed by combination of imaging, serological, histopathological findings; other organ involvement; and steroid responsiveness according to international consensus diagnostic criteria. Type 1 AIP is a pancreatic manifestation of IgG4-related disease, and frequently has elevation of serum IgG4 levels and other IgG4-related diseases. Type 2 AIP is rare in Asia compared to Europe and North America. In contrast to type 1 AIP, type 2 AIP is seen in younger patients, and typical affects males and females equally. Abdominal pain and acute pancreatitis are more frequent in type 2 AIP patients. Although type 1 AIP is rarely associated with inflammatory bowel disease, type 2 AIP frequently has inflammatory bowel disease.

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Abbreviations

AIP:

Autoimmune pancreatitis

LPSP:

Lymphoplasmacytic sclerosing pancreatitis

IgG4:

Immunoglobulin G4

IDCP:

Idiopathic duct-centric chronic pancreatitis

GEL:

Granulocytic epithelial lesion

ICDC:

International consensus diagnostic criteria

CT:

Computed tomography

MRI:

Magnetic resonance imaging

ERCP:

Endoscopic retrograde cholangiopancreatography

MRCP:

Magnetic resonance cholangiopancreatography

IgG4-SC:

IgG4-related sclerosing cholangitis

PSC:

Primary sclerosing cholangitis

IBD:

Inflammatory bowel disease

RF:

Retroperitoneal fibrosis

IgG4-RF:

IgG4-related retroperitoneal fibrosis

ANA:

Antinuclear antigen

HPF:

High power field

UC:

Ulcerative colitis

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Acknowledgement

This chapter was supported by the grant-in-aid for the Refractory Disease from the Ministry of Labor, Health, and Welfare of Japan.

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Authors and Affiliations

  1. Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan

    Go Kuwata M.D. & Terumi Kamisawa M.D., Ph.D.

Authors
  1. Go Kuwata M.D.
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  2. Terumi Kamisawa M.D., Ph.D.
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Corresponding author

Correspondence to Terumi Kamisawa M.D., Ph.D. .

Editor information

Editors and Affiliations

  1. Inflammatory Bowel Disease Center, Department of Gastroenterology and Hepatology, Charité Medical School—Humboldt-University of Berlin, Berlin, Germany

    Daniel C. Baumgart

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Kuwata, G., Kamisawa, T. (2017). Autoimmune Pancreatitis and Other Organ Involvement. In: Baumgart, D. (eds) Crohn's Disease and Ulcerative Colitis. Springer, Cham. https://doi.org/10.1007/978-3-319-33703-6_53

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  • DOI: https://doi.org/10.1007/978-3-319-33703-6_53

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