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Autoimmune Pancreatitis and Other Organ Involvement

  • Go Kuwata
  • Terumi Kamisawa
Chapter

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.

Keywords

Autoimmune pancreatitis Lymphoplasmacytic sclerosing pancreatitis IgG4 Steroid Inflammatory bowel disease Ulcerative colitis 

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

Notes

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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Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of Internal MedicineTokyo Metropolitan Komagome HospitalTokyoJapan

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