Abstract
IgG4-related sclerosing cholangitis (IgG4-SC) is histologically characterized by the inflammation observed around the bile duct epithelium with the epithelium left intact. Transmural inflammation with storiform fibrosis that gives rise to wall thickening is a highly typical histological finding, which may be solely sufficient for diagnosis. Involvement of the connective tissue surrounding the bile ducts is common, and if this involvement is eminent, it appears as a mass (inflammatory pseudotumor). An increase of IgG4-positive cells is a characteristic, but not specific histological feature. In contrast to other diseases that cause an increase of IgG4-positive cells, such as biliary cancers, primary sclerosing cholangitis, and cholelithiasis, the presence of IgG4-positive cells is diffuse, and the IgG4/IgG-positive cell ratio is high. The diagnosis of IgG4-SC with bile duct biopsies is difficult because lesions of IgG4-SC are present in the deep part of the bile duct wall. Liver biopsies may obtain peripheral portal tracts packed with lymphoplasmacytic infiltration, but characteristic bile duct lesions are absent. Thus, the appropriate clinical setting is necessary to make a diagnosis of IgG4-SC with biopsy speciments.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Bartholomew LG, Cain JC, Woolner LB, Utz DC, Ferris DO. Sclerosing cholangitis: its possible association with Riedel’s struma and fibrous retroperitonitis. Report of two cases. N Engl J Med. 1963;269:8–12.
Kawaguchi K, Koike M, Tsuruta K, Okamoto A, Tabata I, Fujita N. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991;22(4):387–95.
Notohara K, Burgart LJ, Yadav D, Chari S, Smyrk TC. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol. 2003;27(8):1119–27.
Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Morimoto H, Miwa A, Uchiyama A, Portmann BC, Nakanuma Y. 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(9):1193–203.
Nakazawa T, Ohara H, Yamada T, Ando H, Sano H, Kajino S, Hashimoto T, Nakamura S, Ando T, Nomura T, Joh T, Itoh M. Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis. Hepatogastroenterology. 2001;48(39):625–30.
Nakazawa T, Ohara H, Sano H, Ando T, Aoki S, Kobayashi S, Okamoto T, Nomura T, Joh T, Itoh M. Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis. Pancreas. 2005;30(1):20–5.
Zen Y, Fujii T, Sato Y, Masuda S, Nakanuma Y. Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4-related disease. Mod Pathol. 2007;20(8):884–94.
Miyabe K, Notohara K, Nakazawa T, Hayashi K, Naitoh I, Okumura F, Shimizu S, Yoshida M, Yamashita H, Takahashi S, Ohara H, Joh T. Histological evaluation of obliterative phlebitis for the diagnosis of autoimmune pancreatitis. J Gastroenterol. 2014;49(4):715–26.
Uehara T, Hamano H, Kawa S, Sano K, Honda T, Ota H. Distinct clinicopathological entity ‘autoimmune pancreatitis-associated sclerosing cholangitis’. Pathol Int. 2005;55(7):405–11.
Graham RP, Smyrk TC, Chari ST, Takahashi N, Zhang L. Isolated IgG4-related sclerosing cholangitis: a report of 9 cases. Hum Pathol. 2014;45(8):1722–9.
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, Kloppel G, Heathcote JG, Khosroshahi A, Ferry JA, Aalberse RC, Bloch DB, Brugge WR, Bateman AC, Carruthers MN, Chari ST, Cheuk W, Cornell LD, Fernandez-Del Castillo C, Forcione DG, Hamilos DL, Kamisawa T, Kasashima S, Kawa S, Kawano M, Lauwers GY, Masaki Y, Nakanuma Y, Notohara K, Okazaki K, Ryu JK, Saeki T, Sahani DV, Smyrk TC, Stone JR, Takahira M, Webster GJ, Yamamoto M, Zamboni G, Umehara H, Stone JH. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181–92.
Harada K, Shimoda S, Kimura Y, Sato Y, Ikeda H, Igarashi S, Ren XS, Sato H, Nakanuma Y. Significance of immunoglobulin G4 (IgG4)-positive cells in extrahepatic cholangiocarcinoma: molecular mechanism of IgG4 reaction in cancer tissue. Hepatology. 2012;56(1):157–64.
Zhang L, Lewis JT, Abraham SC, Smyrk TC, Leung S, Chari ST, Poterucha JJ, Rosen CB, Lohse CM, Katzmann JA, Wu TT. IgG4+ plasma cell infiltrates in liver explants with primary sclerosing cholangitis. Am J Surg Pathol. 2010;34(1):88–94.
Zen Y, Quaglia A, Portmann B. Immunoglobulin G4-positive plasma cell infiltration in explanted livers for primary sclerosing cholangitis. Histopathology. 2011;58(3):414–22.
Oh HC, Kim JG, Kim JW, Lee KS, Kim MK, Chi KC, Kim YS, Kim KH. Early bile duct cancer in a background of sclerosing cholangitis and autoimmune pancreatitis. Intern Med. 2008;47(23):2025–8.
Ohtani H, Ishida H, Ito Y, Yamaguchi T, Koizumi M. Autoimmune pancreatitis and biliary intraepithelial neoplasia of the common bile duct: a case with diagnostically challenging but pathogenetically significant association. Pathol Int. 2011;61(8):481–5.
Naitoh I, Zen Y, Nakazawa T, Ando T, Hayashi K, Okumura F, Miyabe K, Yoshida M, Nojiri S, Kanematsu T, Ohara H, Joh T. Small bile duct involvement in IgG4-related sclerosing cholangitis: liver biopsy and cholangiography correlation. J Gastroenterol. 2011;46(2):269–76.
Deshpande V, Sainani NI, Chung RT, Pratt DS, Mentha G, Rubbia-Brandt L, Lauwers GY. IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. Mod Pathol. 2009;22(10):1287–95.
Nishino T, Oyama H, Hashimoto E, Toki F, Oi I, Kobayashi M, Shiratori K. Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis. J Gastroenterol. 2007;42(7):550–9.
Ohara H, Okazaki K, Tsubouchi H, Inui K, Kawa S, Kamisawa T, Tazuma S, Uchida K, Hirano K, Yoshida H, Nishino T, Ko SB, Mizuno N, Hamano H, Kanno A, Notohara K, Hasebe O, Nakazawa T, Nakanuma Y, Takikawa H. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci. 2012;19(5):536–42.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer Science+Business Media Singapore
About this chapter
Cite this chapter
Notohara, K. (2019). Pathology. In: Kamisawa, T., Kim, MH. (eds) IgG4-Related Sclerosing Cholangitis. Springer, Singapore. https://doi.org/10.1007/978-981-10-4548-6_4
Download citation
DOI: https://doi.org/10.1007/978-981-10-4548-6_4
Published:
Publisher Name: Springer, Singapore
Print ISBN: 978-981-10-4547-9
Online ISBN: 978-981-10-4548-6
eBook Packages: MedicineMedicine (R0)