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Autoimmune Pancreatitis

  • Chapter
Pancreatic Masses

Abstract

Autoimmune pancreatitis (AIP) has been established as a new disease entity of chronic pancreatitis. Although its autoimmune etiology is well established, AIP is still an enigmatic disease. Morphological hallmarks of the disease are narrowing of the pancreatic duct system—and frequently even narrowing of the bile duct—by periductal lymphoplasmocytic inflammation. In cases of more localized inflammation, obstructive jaundice due to a mass-forming lesion in the pancreatic head may mimic pancreatic ductal adenocarcinoma. Therefore, even in tertiary referral centers, AIP patients may have pancreatic surgery for suspected pancreatic cancer due to misclassification.

Histopathologically, the disease can be differentiated as lymphoplasmacytic sclerosing pancreatitis (LPSP) with IgG4-positive cells or idiopathic ductocentric chronic pancreatitis (IDCP) with granulocyte-epithelial lesions (GEL) and eosinophils. Recently, LPSP has been coined AIP type 1 and IDCP as AIP type 2 following a recent consensus conference. Serologically, patients may present with elevated serum IgG and IgG4 levels. Additional autoantibodies have been described targeting ductal and acinar antigens. Association with other autoimmune manifestations in a wide range of organs is a rather frequent finding (other organ involvement, OOI). The most prevalent OOI is immune-associated cholangitis (IAC) in association with AIP type 1. In most cases, autoimmune pancreatitis responds to steroid treatment very quickly, which is of specific importance when considering the differential diagnosis of pancreatic cancer.

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Authors and Affiliations

  1. Division of Surgery, Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institutet, and Centre for Digestive Diseases, Karolinska University Hospital, Stockholm, 141 86, Sweden

    Urban Arnelo & Johannes Matthias Löhr

  2. Division of Pathology, Department of Laboratory Medicine (LABMED), and Department of Clinical Pathology, Karolinska University Hospital, Stockholm, 141 86, Sweden

    Caroline Verbeke

  3. Division of Medical Imaging and Technology, Department of Clinical Science, Intervention and Technology (CLINTEC), Karolinska Institutet, and Department of Radiology, Karolinska University Hospital, Stockholm, 141 86, Sweden

    Nikolaos Kartalis

Authors
  1. Urban Arnelo
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  2. Caroline Verbeke
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  3. Nikolaos Kartalis
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  4. Johannes Matthias Löhr
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Corresponding author

Correspondence to Urban Arnelo .

Editor information

Editors and Affiliations

  1. Division of Gastroenterology, University of Florida, Gainesville, Florida, USA

    Mihir S. Wagh

  2. Division of Gastroenterology, University of Florida, Gainesville, Florida, USA

    Peter V. Draganov

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© 2016 Springer International Publishing Switzerland

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Arnelo, U., Verbeke, C., Kartalis, N., Löhr, J.M. (2016). Autoimmune Pancreatitis. In: Wagh, M., Draganov, P. (eds) Pancreatic Masses. Springer, Cham. https://doi.org/10.1007/978-3-319-19677-0_5

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  • DOI: https://doi.org/10.1007/978-3-319-19677-0_5

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-19676-3

  • Online ISBN: 978-3-319-19677-0

  • eBook Packages: MedicineMedicine (R0)

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