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


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...


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.



Autoimmune pancreatitis


Autoimmune pancreatocholangitis


Antinuclear antibodies


IgG4-associated cholangitis




High-power field


Magnetic resonance cholangiopancreatography


Primary sclerosing cholangitis



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.


  1. 1.
    Sarles H, Sarles JC, Muratore R, Guien C (1961) Chronic inflammatory sclerosis of the pancreas—an autonomous pancreatic disease? Am J Dig Dis 6:688–698PubMedCrossRefGoogle Scholar
  2. 2.
    Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ et al (2010) Differences in clinical profile and relapse rate of type 1 vs type 2 autoimmune pancreatitis. Gastroenterology 139:140–148PubMedCrossRefGoogle Scholar
  3. 3.
    Esposito I, Born D, Bergmann F, Longerich T, Welsch T, Giese NA et al (2008) Autoimmune pancreatocholangitis, non-autoimmune pancreatitis and primary sclerosing cholangitis: a comparative morphological and immunological analysis. PLoS One 3:e2539PubMedCrossRefGoogle Scholar
  4. 4.
    Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ et al (2008) Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 134:706–715PubMedCrossRefGoogle Scholar
  5. 5.
    Webster GJ, Pereira SP, Chapman RW (2009) Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis-overlapping or separate diseases? J Hepatol 51:398–402PubMedCrossRefGoogle Scholar
  6. 6.
    Alderlieste YA, Van den Elzen BD, Rauws EA, Beuers U (2009) Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. Digestion 79:220–228PubMedCrossRefGoogle Scholar
  7. 7.
    Umemura T, Zen Y, Hamano H, Kawa S, Nakanuma Y, Kiyosawa K (2007) Immunoglobin G4-hepatopathy: association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology 46:463–471PubMedCrossRefGoogle Scholar
  8. 8.
    Bergquist A, Ekbom A, Olsson R, Kornfeldt D, Lööf L, Danielsson A et al (2002) Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 36:321–327PubMedCrossRefGoogle Scholar
  9. 9.
    Oh HC, Kim JG, Kim JW, Lee KS, Kim MK, Chi KC et al (2008) Early bile duct cancer in a background of sclerosing cholangitis and autoimmune pancreatitis. Intern Med 47:2025–2028PubMedCrossRefGoogle Scholar
  10. 10.
    Cheung MT, Lo IL (2008) IgG4-related sclerosing lymphoplasmacytic pancreatitis and cholangitis mimicking carcinoma of pancreas and Klatskin tumour. ANZ J Surg 78:252–256PubMedCrossRefGoogle Scholar
  11. 11.
    Inoue H, Miyatani H, Sawada Y, Yoshida Y (2006) A case of pancreas cancer with autoimmune pancreatitis. Pancreas 33:208–209PubMedCrossRefGoogle Scholar
  12. 12.
    Witkiewicz AK, Kennedy EP, Kennyon L, Yeo CJ, Hruban RH (2008) Synchronous autoimmune pancreatitis and infiltrating pancreatic ductal adenocarcinoma: case report and review of the literature. Hum Pathol 39:1548–1551PubMedCrossRefGoogle Scholar
  13. 13.
    Gill J, Angelo N, Yeong ML, McIvor N (2009) Salivary duct carcinoma arising in IgG4-related autoimmune disease of the parotid gland. Hum Pathol 40:881–886PubMedCrossRefGoogle Scholar
  14. 14.
    Hsing AW, McLaughlin JK, Olsen JH, Mellemkjar L, Wacholder S, Fraumeni JF Jr (1995) Cancer risk following primary hemochromatosis: a population-based cohort study in Denmark. Int J Cancer 60:160–162PubMedCrossRefGoogle Scholar
  15. 15.
    Zhang L, Lewis JT, Abraham SC, Smyrk TC, Leung S, Chari ST et al (2010) IgG4+ plasma cell infiltrates in liver explants with primary sclerosing cholangitis. Am J Surg Pathol 34:88–94PubMedCrossRefGoogle Scholar

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

Personalised recommendations