Histologic Features

  • Günter Klöppel
  • Thomas C. Smyrk


Autoimmune pancreatitis (AIP) currently encompasses two conditions: type 1 AIP, which is the pancreatic manifestation of a multiorgan IgG4-related disease, and type 2 AIP, which is limited to the pancreas. The two types share common histologic features such as periductal lymphoplasmacytic inflammation, loss of pancreatic parenchyma, and increased fibrosis, but there are morphologic differences which often allow one to distinguish between the two. Type 1 AIP is more likely to have areas of storiform fibrosis and is much more likely to have lymphocytic phlebitis. There are almost always increased numbers of IgG4-positive plasma cells. Type 2 AIP is more likely to have active (neutrophil) inflammation, particularly in duct epithelium; these lesions are termed “granulocytic epithelial lesions” and are a very helpful diagnostic finding. Type 2 AIP seldom has increased tissue IgG4. Both types of AIP can be mistaken clinically for malignancy. The diagnosis can often be made on large-caliber trucut biopsy.


Autoimmune Pancreatitis IgG4 Serum Level Distal Bile Duct Lymphoplasmacytic Infiltrate Storiform Fibrosis 
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.


  1. 1.
    Kloppel G. Chronic pancreatitis, pseudotumors and other tumor-like lesions. Mod Pathol. 2007;20 Suppl 1:S113–31.PubMedCrossRefGoogle Scholar
  2. 2.
    Chari ST, Kloeppel G, Zhang L, et al. Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas. 2010;39:549–54.PubMedCrossRefGoogle Scholar
  3. 3.
    Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.Google Scholar
  4. 4.
    Chandan VS, Iacobuzio-Donahue C, Abraham SC. Patchy distribution of pathologic abnormalities in autoimmune pancreatitis: implications for preoperative diagnosis. Am J Surg Pathol. 2008;32:1762–9.PubMedCrossRefGoogle Scholar
  5. 5.
    Abraham SC, Leach S, Yeo CJ, et al. Eosinophilic pancreatitis and increased eosinophils in the pancreas. Am J Surg Pathol. 2003;27:334–42.PubMedCrossRefGoogle Scholar
  6. 6.
    Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–4.PubMedCrossRefGoogle Scholar
  7. 7.
    Zhang L, Notohara K, Levy MJ, et al. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol. 2007;20:23–8.PubMedCrossRefGoogle Scholar
  8. 8.
    Dhall D, Suriawinata AA, Tang LH, et al. Use of immunohistochemistry for IgG4 in the distinction of autoimmune pancreatitis from peritumoral pancreatitis. Hum Pathol. 2010;41:643–52.PubMedCrossRefGoogle Scholar
  9. 9.
    Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34:1812–9.PubMedCrossRefGoogle Scholar
  10. 10.
    Shrestha B, Sekiguchi H, Colby TV, et al. Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis: report of 6 and 12 cases with similar histopathology. Am J Surg Pathol. 2009;33:1450–62.PubMedCrossRefGoogle Scholar
  11. 11.
    Zen Y, Inoue D, Kitao A, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2009;33:1886–93.PubMedCrossRefGoogle Scholar
  12. 12.
    Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17:303–32.PubMedCrossRefGoogle Scholar
  13. 13.
    van Heerde MJ, Biermann K, Zondervan PE, et al. Prevalence of autoimmune pancreatitis and other benign disorders in pancreatoduodenectomy for presumed malignancy of the pancreatic head. Dig Dis Sci. 2012;57(9):2458–65.Google Scholar
  14. 14.
    Chu KE, Papouchado BG, Lane Z, et al. The role of Movat pentachrome stain and immunoglobulin G4 immunostaining in the diagnosis of autoimmune pancreatitis. Mod Pathol. 2009;22:351–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Deshpande V, Mino-Kenudson M, Brugge WR, et al. Endoscopic ultrasound guided fine needle aspiration biopsy of autoimmune pancreatitis: diagnostic criteria and pitfalls. Am J Surg Pathol. 2005;29:1464–71.PubMedCrossRefGoogle Scholar
  16. 16.
    Holmes BJ, Hruban RH, Wolfgang CL, et al. Fine needle aspirate of autoimmune pancreatitis (lymphoplasmacytic sclerosing pancreatitis): cytomorphologic characteristics and clinical correlates. Acta Cytol. 2012;56:228–32.PubMedCrossRefGoogle Scholar
  17. 17.
    Zamboni GLJ, Capelli P. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study of 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004;445:552–63.PubMedCrossRefGoogle Scholar
  18. 18.
    Detlefsen S, Mohr Drewes A, Vyberg M, et al. Diagnosis of autoimmune pancreatitis by core needle biopsy: application of six microscopic criteria. Virchows Arch. 2009;454:531–9.PubMedCrossRefGoogle Scholar
  19. 19.
    Notohara K, Burgart LJ, Yadav D, et al. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol. 2003;27:1119–27.PubMedCrossRefGoogle Scholar
  20. 20.
    Kojima M, Sipos B, Klapper W, et al. Autoimmune pancreatitis: frequency, IgG4 expression, and clonality of T and B cells. Am J Surg Pathol. 2007;31:521–8.PubMedCrossRefGoogle Scholar
  21. 21.
    Kamisawa T, Okamoto A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol. 2006;41:613–25.PubMedCrossRefGoogle Scholar
  22. 22.
    Kamisawa T, Chari ST, Giday SA, et al. Clinical profile of autoimmune pancreatitis and its histological subtypes: an international multicenter survey. Pancreas. 2011;40:809–14.PubMedCrossRefGoogle Scholar
  23. 23.
    Kloppel G, Detlefsen S, Chari ST, et al. Autoimmune pancreatitis: the clinicopathological characteristics of the subtype with granulocytic epithelial lesions. J Gastroenterol. 2010;45:787–93.PubMedCrossRefGoogle Scholar
  24. 24.
    Ito T, Nishimori I, Inoue N, et al. Treatment for autoimmune pancreatitis: consensus on the treatment for patients with autoimmune pancreatitis in Japan. J Gastroenterol. 2007;42 Suppl 18:50–8.PubMedCrossRefGoogle Scholar
  25. 25.
    Saito T, Tanaka S, Yoshida H, et al. A case of autoimmune pancreatitis responding to steroid therapy. Evidence of histologic recovery. Pancreatology. 2002;2:550–6.PubMedCrossRefGoogle Scholar
  26. 26.
    Chari ST, Murray JA. Autoimmune pancreatitis, Part II: the relapse. Gastroenterology. 2008;134:625–8.PubMedCrossRefGoogle Scholar
  27. 27.
    Detlefsen S, Zamboni G, Frulloni L, et al. Clinical features and relapse rates after surgery in type 1 autoimmune pancreatitis differ from type 2: a study of 114 surgically treated European patients. Pancreatology. 2012;12:276–83.PubMedGoogle Scholar
  28. 28.
    Taniguchi T, Okazaki K, Okamoto M, et al. High prevalence of autoantibodies against carbonic anhydrase II and lactoferrin in type 1 diabetes: concept of autoimmune exocrinopathy and endocrinopathy of the pancreas. Pancreas. 2003;27:26–30.PubMedCrossRefGoogle Scholar
  29. 29.
    Lohr JM, Faissner R, Koczan D, et al. Autoantibodies against the exocrine pancreas in autoimmune pancreatitis: gene and protein expression profiling and immunoassays identify pancreatic enzymes as a major target of the inflammatory process. Am J Gastroenterol. 2010;105:2060–71.PubMedCrossRefGoogle Scholar
  30. 30.
    Deshpande V, Chicano S, Finkelberg D, et al. Autoimmune pancreatitis: a systemic immune complex mediated disease. Am J Surg Pathol. 2006;30:1537–45.PubMedCrossRefGoogle Scholar
  31. 31.
    Detlefsen S, Brasen JH, Zamboni G, et al. Deposition of complement C3c, immunoglobulin (Ig)G4 and IgG at the basement membrane of pancreatic ducts and acini in autoimmune pancreatitis. Histopathology. 2010;57:825–35.PubMedCrossRefGoogle Scholar
  32. 32.
    Inoue H, Miyatani H, Sawada Y, et al. A case of pancreas cancer with autoimmune pancreatitis. Pancreas. 2006;33:208–9.PubMedCrossRefGoogle Scholar
  33. 33.
    Witkiewicz AK, Kennedy EP, Kennyon L, et al. Synchronous autoimmune pancreatitis and infiltrating pancreatic ductal adenocarcinoma: case report and review of the literature. Hum Pathol. 2008;39:1548–51.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of PathologyUniversity Hospital, University of KielMunichGermany
  2. 2.Department of PathologyMayo ClinicRochesterUSA

Personalised recommendations