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Diagnostic Criteria

  • Hirotaka OharaEmail author
  • Itaru Naitoh
  • Kazuki Hayashi
  • Katsuyuki Miyabe
  • Takahiro Nakazawa
Chapter
First Online:

Abstract

As IgG4-related sclerosing cholangitis (IgG4-SC) exhibits various cholangiographic features similar to those of primary sclerosing cholangitis (PSC), pancreatic cancer, and cholangiocarcinoma, the differential diagnosis of IgG4-SC from these three progressive or malignant diseases is very important. In the criteria of Japanese group, diagnosis of IgG4-SC is based on the following four criteria: characteristic biliary imaging findings, elevation of serum IgG4 levels, coexistence of IgG4-related diseases, except those of the biliary tract, and characteristic histopathologic features. IgG4-SC is frequently associated with autoimmune pancreatitis (AIP), and this association is useful for diagnosis of IgG4-SC. However, it is particularly difficult to accurately diagnose IgG4-SC in the absence of AIP. Evaluation of the effectiveness of steroid therapy is an optional extra diagnostic criterion to confirm a diagnosis of IgG4-SC.

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References

  1. 1.
    Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732–8.CrossRefPubMedGoogle Scholar
  2. 2.
    Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, et al. IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis? Am J Surg Pathol. 2004;28:1193–203.CrossRefPubMedGoogle Scholar
  3. 3.
    Nakazawa T, Ohara H, Yamada T, Ando H, Sano H, Kajino S, et al. Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis. Hepato-Gastroenterology. 2001;48:625–30.PubMedGoogle Scholar
  4. 4.
    Nakazawa T, Ohara H, Sano H, Ando T, Aoki S, Kobayashi S, et al. Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis. Pancreas. 2005;30:20–5.PubMedGoogle Scholar
  5. 5.
    Nishino T, Toki F, Oyama H, Oi I, Kobayashi M, Takasaki K, et al. Biliary tract involvement in autoimmune pancreatitis. Pancreas. 2005;30:76–82.PubMedGoogle Scholar
  6. 6.
    Hirano K, Tada M, Isayama H, Yagioka H, Sasaki T, Kogure H, et al. Long-term prognosis of autoimmune pancreatitis with and without corticosteroid treatment. Gut. 2007;56:1719–24.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–15.CrossRefPubMedGoogle Scholar
  8. 8.
    Nakazawa T, Ohara H, Sano H, Aoki S, Kobayashi S, Okamoto T, et al. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointest Endosc. 2004;60:937–44.CrossRefPubMedGoogle Scholar
  9. 9.
    Nishino T, Oyama H, Hashimoto E, Toki F, Oi I, Kobayashi M, et al. Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis. J Gastroenterol. 2007;42:550–9.CrossRefPubMedGoogle Scholar
  10. 10.
    Kalaitzakis E, Levy M, Kamisawa T, Johnson GJ, Baron TH, Topazian MD, et al. Endoscopic retrograde cholangiography does not reliably distinguish IgG4-associated cholangitis from primary Sclerosing cholangitis or cholangiocarcinoma. Clin Gastroenterol Hepatol. 2011;9:800–3.e2.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Nakazawa T, Ando T, Hayashi K, Naitoh I, Okumura F, Miyabe K, et al. Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholanigiographic classification. J Gastroenterol. 2012;40:79–87.CrossRefGoogle Scholar
  12. 12.
    Ohara H, Okazaki K, Tsubouchi H, Inui K, Kawa S, Kamisawa T, et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci. 2012;19:536–42.CrossRefPubMedGoogle Scholar
  13. 13.
    Nakazawa T, Ohara H, Sano H, Ando T, Joh T. Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas. 2006;32:229.CrossRefPubMedGoogle Scholar
  14. 14.
    Hirano K, Tada M, Isayama H, Yamamoto K, Mizuno S, Yagioka H, et al. Endoscopic evaluation of factors contributing to intrapancreatic biliary stricture in autoimmune pancreatitis. Gastrointest Endosc. 2010;71:85–90.CrossRefPubMedGoogle Scholar
  15. 15.
    Koyama R, Imamura T, Okuda C, Sakamoto N, Honjo H, Takeuchi K, et al. Ultrasonographic imaging of bile duct lesions in autoimmune pancreatitis. Pancreas. 2008;37:259–64.CrossRefPubMedGoogle Scholar
  16. 16.
    Itoh S, Nagasaka T, Suzuki K, Satake H, Ota T, Naganawa N. Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings. Clin Radiol. 2009;64:1104–14.CrossRefPubMedGoogle Scholar
  17. 17.
    Naitoh I, Nakazawa T, Ohara H, Andoh T, Hayashi K, Tanaka H, et al. Endoscopic transpapillary intraductal ultrasonography and biopsy in the diagnosis of IgG4-related sclerosing cholangitis. J Gastroenterol. 2009;44:1147–55.CrossRefPubMedGoogle Scholar
  18. 18.
    Naitoh I, Nakazawa T, Hayashi K, Miyabe K, Shimizu S, Kondo H, et al. Comparison of intraductal ultrasonography findings between primary sclerosing cholangitis and IgG4-related sclerosing cholangitis. J Gastroenterol Hepatol. 2015;30:1104–9.CrossRefPubMedGoogle Scholar
  19. 19.
    Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol. 2006;101:2070–5.CrossRefPubMedGoogle Scholar
  20. 20.
    Oseini AM, Chaiteerakij R, Shire AM, Ghazale A, Kaiya J, Moser CD, et al. Utility of serum immunoglobulin G4 in distinguishing immunoglobulin G4-associated cholangitis from Cholangiocarcinoma. Hepatology. 2011;54:940–8.CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Ohara H, Nakazawa T, Kawa S, Kamisawa T, Shimosegawa T, Uchida K, et al. Establishment of a serum IgG4 cut-off value for the differential diagnosis of IgG4-related sclerosing cholangitis: a Japanese cohort. J Gastroenterol Hepatol. 2013;28:1247–51.CrossRefPubMedGoogle Scholar
  22. 22.
    Hamano H, Kawa S, Uehara T, Ochi Y, Takayama M, Komatsu K, et al. Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointest Endosc. 2005;62:152–7.CrossRefPubMedGoogle Scholar
  23. 23.
    Graham R, Smyrk T, Chari S, Takahashi N, Zhang L. Isolated IgG4-related sclerosing cholangitis: a report of 9 cases. Hum Pathol. 2014;45:1722–9.CrossRefPubMedGoogle Scholar
  24. 24.
    Nakazawa T, Ikeda Y, Kawaguchi Y, Kitagawa H, Takada H, Takeda Y, et al. Isolated intrapacreatic IgG4-related sclerosing cholangitis. World J Gastroenterol. 2015;21:1334–43.CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–4.CrossRefPubMedGoogle Scholar
  26. 26.
    Ohara H, Nakazawa T, Sano H, Ando T, Okamoto T, Takada H, et al. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. Pancreas. 2005;31:232–7.CrossRefPubMedGoogle Scholar
  27. 27.
    Hamano H, Arakura N, Muraki T, Ozaki Y, Kiyosawa K, Kawa S. Prevalence and distribution of extrapancreatic lesions complicating autoimmune pancreatitis. J Gastroenterol. 2006;41:1197–205.CrossRefPubMedGoogle Scholar
  28. 28.
    Naitoh I, Nakazawa T, Ohara H, Andoh T, Hayashi K, Tanaka H, et al. Clinical significance of extrapancreatic lesions in autoimmune pancreatitis. Pancreas. 2010;39:e1–5.CrossRefPubMedGoogle Scholar
  29. 29.
    Sano H, Nakazawa T, Ando T, Hayashi K, Naitoh I, Okumura F, Miyabe K, Yoshida M, Takahashi S, Ohara H, Joh T. Clinical characteristics of inflammatory bowel disease associated with primary sclerosing cholangitis. J Hepatobiliary Pancreat Sci. 2011;18:154–61.CrossRefPubMedGoogle Scholar
  30. 30.
    Nakanuma Y, Zen Y. Pathology and immunopathology of immunoglobulin G4-related sclerosing cholangitis: the latest addition to the sclerosing cholangitis family. Hepatol Res. 2007;37(Suppl 3):S478–86.CrossRefPubMedGoogle Scholar
  31. 31.
    Zhang L, Lewis JT, Abraham SC, Smyrk TC, Leung S, Chari ST. IgG4+ plasma cell infiltrates in liver explants with primary sclerosing cholangitis. Am J Surg Pathol. 2010;34:88–94.CrossRefPubMedGoogle Scholar
  32. 32.
    Kubota K, Kato S, Akiyama T, Yoneda M, Fujita K, Ogawa M. Differentiating sclerosing cholangitis caused by autoimmune pancreatitis and primary sclerosing cholangitis according to endoscopic duodenal papillary features. Gastrointest Endosc. 2008;68:1204–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Umemura T, Zen Y, Hamano H, Kawa S, Nakanuma Y, Kiyosawa K, et al. Immunoglobulin G4-hepatopathy: association of immunoglobulin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology. 2007;46:463–71.CrossRefPubMedGoogle Scholar
  34. 34.
    Naitoh I, Zen Y, Nakazawa T, Ando T, Hayashi K, Okumura F, et al. Small bile duct involvement in IgG4-related sclerosing cholangitis: liver biopsy and cholangiography correlation. J Gastroenterol. 2011;46:269–76.CrossRefPubMedGoogle Scholar
  35. 35.
    Tomiyama T, Uchida K, Matsushita M, Ikeura T, Fukui T, Takaoka M, et al. Comparison of steroid pulse therapy and conventional oral steroid therapy as initial treatment for autoimmune pancreatitis. J Gastroenterol. 2011;46:696–704.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media Singapore 2019

Authors and Affiliations

  • Hirotaka Ohara
    • 1
    Email author
  • Itaru Naitoh
    • 2
  • Kazuki Hayashi
    • 2
  • Katsuyuki Miyabe
    • 2
  • Takahiro Nakazawa
    • 2
  1. 1.Department of Community-Based Medical EducationNagoya City University Graduate School of Medical SciencesNagoyaJapan
  2. 2.Department of Gastroenterology and MetabolismNagoya City University Graduate School of Medical SciencesNagoyaJapan

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