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Virchows Archiv

, Volume 458, Issue 6, pp 761–765 | Cite as

IgG4-associated cholangitis with cholangiocarcinoma

  • Beate K. Straub
  • Irene Esposito
  • Daniel Gotthardt
  • Boris Radeleff
  • Dalibor Antolovic
  • Christa Flechtenmacher
  • Peter Schirmacher
Case Report

Introduction

Autoimmune pancreatitis (AIP) was first described by Sarles and coauthors [1] as a primary inflammatory sclerosis of the pancreas and is currently subclassified into two clinical entities named type 1 and type 2 AIP [2]. Clinical features of type 1 AIP include elevated IgG4-levels in the serum and excellent response to steroids, whereas in type 2 AIP, IgG4 levels are not elevated. Histologic criteria for type 1 AIP include periductal lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative venulitis, whereas the hallmark of type 2 AIP is the presence of epithelial granulocytic lesions. Extrapancreatic disease occurs in more than 45% of patients with AIP, and especially intra- and extrahepatic biliary tree involvement has been described as a peculiar form of sclerosing cholangitis [3, 4, 5]. Less frequently, the disease may only involve the intra- and/or extrahepatic bile ducts, then termed IgG4-associated cholangitis (IAC) [6]. Currently, IAC and AIP are seen as...

Keywords

Bile Duct Cholangiocarcinoma Primary Sclerosing Cholangitis Extrahepatic Bile Duct Intrahepatic Cholangiocarcinoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Abbreviations

AIP

Autoimmune pancreatitis

AIPC

Autoimmune pancreatocholangitis

ANA

Antinuclear antibodies

IAC

IgG4-associated cholangitis

CC

Cholangiocarcinoma

HPF

High-power field

MRCP

Magnetic resonance cholangiopancreatography

PSC

Primary sclerosing cholangitis

Notes

Acknowledgment

BKS was supported by a stipend of the Olympia-Morata program of the Medical Faculty of Heidelberg University.

Disclosure statement

The authors declare that no financial or other conflict of interest exists in relation to the content of the article.

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Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Beate K. Straub
    • 1
  • Irene Esposito
    • 2
    • 3
  • Daniel Gotthardt
    • 4
  • Boris Radeleff
    • 5
  • Dalibor Antolovic
    • 6
  • Christa Flechtenmacher
    • 1
  • Peter Schirmacher
    • 1
  1. 1.Institute of PathologyUniversity HospitalHeidelbergGermany
  2. 2.Institute of PathologyTechnische Universität MünchenMunichGermany
  3. 3.Institute of PathologyHelmholtz Zentrum MünchenNeuherbergGermany
  4. 4.Department of Internal Medicine IVUniversity HospitalHeidelbergGermany
  5. 5.Department of Diagnostic and Interventional RadiologyUniversity HospitalHeidelbergGermany
  6. 6.Department of SurgeryUniversity HospitalHeidelbergGermany

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