Abstract
Background
IgG4-related sclerosing cholangitis (IgG4-SC) needs to be differentiated from primary sclerosing cholangitis (PSC). In this study, we performed a retrospective study to reveal cases in which liver needle biopsy was useful for differential diagnosis.
Methods
Nineteen patients with IgG4-SC and 22 patients with PSC were studied. All patients underwent endoscopic retrograde cholangiography and liver needle biopsy. We defined small bile duct involvement of IgG4-SC histologically as damage to the small bile duct associated with infiltration of ≥10 IgG4+ plasma cells per high power field (HPF). Clinicopathological characteristics were compared between IgG4-SC patients with and without small bile duct involvement.
Results
Small bile duct involvement was observed in 5 (26%) of the patients with IgG4-SC. Patients with small bile duct involvement showed a higher incidence of intrahepatic biliary strictures on cholangiography (80 vs. 21%, p = 0.038). Conversely, 4 of 7 (57%) patients with intrahepatic biliary strictures on cholangiography had histologically evident small duct involvement. The number of IgG4+ plasma cells was significantly correlated with the site of the most proximal stricture on cholangiograms (p = 0.021). The number of IgG4+ plasma cells per HPF was significantly higher in IgG4-SC patients with intrahepatic biliary strictures than in those with PSC (13.4 vs. 0.4 cells/HPF, p < 0.001).
Conclusions
Involvement of small bile ducts is more frequent in patients with intrahepatic biliary strictures on cholangiography, and liver needle biopsy is especially useful for these patients.
Similar content being viewed by others
References
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.
Nakazawa T, Ohara H, Ando T, Hayashi K, Naitoh I, Okumura F, et al. Clinical course and indications for steroid therapy of sclerosing cholangitis associated with autoimmune pancreatitis. Hepatogastroenterology. 2009;56:584–8.
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.
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.
Hayashi K, Nakazawa T, Ohara H, Ando T, Takada H, Tanaka H, et al. Autoimmune sclerosing cholangiopancreatitis with little pancreatic involvements by imaging findings. Hepatogastroenterology. 2007;54:2146–51.
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.
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.
Nishino T, Oyama H, Hashimoto E, Toki S, Oi I, Kobayashi M, et al. Clinicopathological differentiation between sclerosing cholangitis with autoimmune pancreatitis and primary sclerosing cholangitis. J Gastroenterol. 2007;42:550–9.
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.
Kubota K, Kato S, Akiyama T, Yoneda M, Fujita K, Ogawa M, et al. Differentiating sclerosing cholangitis caused by autoimmune pancreatitis and primary sclerosing cholangitis according to endoscopic duodenal papillary features. Gastrointest Endosc. 2008;68:1204–8.
Naitoh I, Nakazawa T, Ohara H, Ando 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.
Umemura T, Zen Y, Hamano H, Kawa S, Nakanuma Y, Kiyosawa K. Immunoglobin G4-hepatopathy: association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis. Hepatology. 2007;46:463–71.
Deshpande V, Sainani NI, Chung RT, Pratt DS, Mentha G, Rubbia-Brandt L, et al. IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. Mod Pathol. 2009;22:1287–95.
Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol. 2006;41:626–31.
Otsuki M, Chung JB, Okazaki K, Kim MH, Kamisawa T, Kawa S, et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan–Korea symposium on Autoimmune Pancreatitis. J Gastroenterol. 2008;43:403–8.
Lindor KD, LaRusso NF. Primary sclerosing cholangitis. In: Schiff ER, Sorrell MF, Maddrey WC, editors. Disease of the liver. 9th ed. Philadelphia: JB Lippincott; 2003. p. 673–84.
Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol. 2006;4:1010–6.
Nakazawa T, Ohara H, Sano H, Ando T, Joh T. Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas. 2006;32:229.
Zhang L, Lewis JT, Abraham SC, Smyrk TC, Leung S, Chari ST, et al. IgG4+ plasma cell infiltrates in liver explants with primary sclerosing cholangitis. Am J Surg Pathol. 2010;34:88–94.
Acknowledgments
This study was supported by the Pancreas Research Foundation of Japan and by Health and Labor Sciences research grants (Research on Specific Diseases, Intractable Diseases of the Pancreas, Ministry of Health Labor and Welfare, Japan).
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Naitoh, I., Zen, Y., Nakazawa, T. et al. Small bile duct involvement in IgG4-related sclerosing cholangitis: liver biopsy and cholangiography correlation. J Gastroenterol 46, 269–276 (2011). https://doi.org/10.1007/s00535-010-0319-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00535-010-0319-0