Skip to main content
Top
Published in: Abdominal Radiology 6/2019

01-06-2019 | Autoimmune Pancreatitis | Hepatobiliary

Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center

Authors: Jordan Swensson, Temel Tirkes, Mark Tann, Enming Cui, Kumaresan Sandrasegaran

Published in: Abdominal Radiology | Issue 6/2019

Login to get access

Abstract

Purpose

To compare the cross-sectional imaging findings of immunoglobulin G4-related sclerosing cholangiopathy (IgG4-SC) and cholangiocarcinoma (CCA).

Methods

Retrospective search of radiology and pathology databases identified 24 patients with IgG4-SC and over 500 patients with CCA from January 2009 to December 2016. Patients with no pre-treatment imaging studies available on PACS, non-contrasted imaging only, presence of mass lesions, metastatic disease or biliary stents were excluded. 17 patients with IgG4-SC and a selected group of 20 (age and gender matched) patients with CCA were obtained. Images were blinded and independently reviewed by two radiologists. Differences in proportions and means between groups were analyzed using Fishers and Mann–Whitney tests, respectively.

Results

Both readers identified a statistically significant difference in the presence of abrupt common bile duct narrowing between IgG4-SC and CCA (6.7% vs. 68.4%, p < 0.001; 33.3% vs. 75%, p = 0.019). No difference was seen in biliary wall thickening, wall enhancement, extrahepatic exclusive location of disease, or pancreatic duct dilation. Inter-observer variability was κ = 0.52. Total bilirubin and CA 19-9 were unable to differentiate between IgG4-SC and CCA. Serum IgG4 was positive in two of six IgG4-SC patients who were tested.

Conclusion

IgG4-SC and CCA share many clinical and imaging findings on CT and MRI. Abrupt bile duct cut sign strongly favors CCA. In the absence of this finding, IgG4-SC should be considered in the differential diagnosis in all cases of suspected extrahepatic CCA.
Literature
1.
go back to reference Katabathina V, Khalil S, Shin S, Lath N, Menias CO, Prasad SR (2016) Immunoglobulin G4-Related Disease: Recent Advances in Pathogenesis and Imaging Findings. Radiol Clin N Am 54:535–551CrossRefPubMed Katabathina V, Khalil S, Shin S, Lath N, Menias CO, Prasad SR (2016) Immunoglobulin G4-Related Disease: Recent Advances in Pathogenesis and Imaging Findings. Radiol Clin N Am 54:535–551CrossRefPubMed
2.
go back to reference Vlachou P, Khalili K, Jang HJ, Fischer S, Hirschfield GM, Kim TK (2011) IgG4-related Sclerosing Disease: Autoimmune Pancreatitis and Extrapancreatic Manifestations. Radiographics 31:1379–1402CrossRefPubMed Vlachou P, Khalili K, Jang HJ, Fischer S, Hirschfield GM, Kim TK (2011) IgG4-related Sclerosing Disease: Autoimmune Pancreatitis and Extrapancreatic Manifestations. Radiographics 31:1379–1402CrossRefPubMed
3.
go back to reference Al Zahrani H, Kyoung KT, Khalili K, Vlachou P, Yu H, Jang HJ (2014) IgG4-Related Disease in the Abdomen: A Great Mimicker. Seminars in Ultrasound CT MRI 35:240–254CrossRef Al Zahrani H, Kyoung KT, Khalili K, Vlachou P, Yu H, Jang HJ (2014) IgG4-Related Disease in the Abdomen: A Great Mimicker. Seminars in Ultrasound CT MRI 35:240–254CrossRef
4.
go back to reference Itoh S, Nagasaka T, Suzuki K, Ota T, Naganawa S (2009) Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings. Clinical Radiology 64:1104–1114CrossRefPubMed Itoh S, Nagasaka T, Suzuki K, Ota T, Naganawa S (2009) Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings. Clinical Radiology 64:1104–1114CrossRefPubMed
5.
go back to reference Hamano H, Kawa S, Uehara T, et al (2005) Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointestinal Endoscopy 62(1): 152–157CrossRefPubMed Hamano H, Kawa S, Uehara T, et al (2005) Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis? Gastrointestinal Endoscopy 62(1): 152–157CrossRefPubMed
6.
go back to reference Kim JH, Byun JH, Kim SY et al (2013), Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI. Acta Radiologica 54:601–607CrossRefPubMed Kim JH, Byun JH, Kim SY et al (2013), Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI. Acta Radiologica 54:601–607CrossRefPubMed
7.
go back to reference Olsson R, Glaumann H, Almer S et al (2009) High prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis. European Journal of Internal Medicine 20:190–196CrossRefPubMed Olsson R, Glaumann H, Almer S et al (2009) High prevalence of small duct primary sclerosing cholangitis among patients with overlapping autoimmune hepatitis and primary sclerosing cholangitis. European Journal of Internal Medicine 20:190–196CrossRefPubMed
8.
go back to reference Oh HC, Kim MH, Lee KT et al (2010) Clinical clues to suspicion of IgG4-associated sclerosing cholangitis disguised as primary sclerosing cholangitis or hilar cholangiocarcinoma. Journal of Gastroenterology and Hepatology 25:1831–1837CrossRefPubMed Oh HC, Kim MH, Lee KT et al (2010) Clinical clues to suspicion of IgG4-associated sclerosing cholangitis disguised as primary sclerosing cholangitis or hilar cholangiocarcinoma. Journal of Gastroenterology and Hepatology 25:1831–1837CrossRefPubMed
9.
go back to reference Deshpande V, Sainani NI, Chung RT et al (2009) IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. Modern Pathology 22:1287–1295CrossRefPubMed Deshpande V, Sainani NI, Chung RT et al (2009) IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. Modern Pathology 22:1287–1295CrossRefPubMed
10.
go back to reference Sivakumaran Y, Le Page PA, Becerril-Martinez G et al (2014) IgG4-related sclerosing cholangitis: the cholangiocarcinoma mimic. ANZ J Surg 84:486–487CrossRefPubMed Sivakumaran Y, Le Page PA, Becerril-Martinez G et al (2014) IgG4-related sclerosing cholangitis: the cholangiocarcinoma mimic. ANZ J Surg 84:486–487CrossRefPubMed
11.
go back to reference Ghazale A, Chari S, Smyrk T, Takahashi N, Levy M (2008) Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy. Gastroenterology 134:706–715CrossRefPubMed Ghazale A, Chari S, Smyrk T, Takahashi N, Levy M (2008) Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy. Gastroenterology 134:706–715CrossRefPubMed
12.
go back to reference Khosroshahi A, Stone JH (2011) A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol 23(1):57–66CrossRefPubMed Khosroshahi A, Stone JH (2011) A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol 23(1):57–66CrossRefPubMed
14.
go back to reference Soares KC, Kamel I, Cosgrove DP, Herman JM, Pawlik TM (2014) Hilar cholangiocarcinoma: diagnosis, treatment options, and management. Hepatobiliary Surg Nutr. 3(1): 18–34PubMedPubMedCentral Soares KC, Kamel I, Cosgrove DP, Herman JM, Pawlik TM (2014) Hilar cholangiocarcinoma: diagnosis, treatment options, and management. Hepatobiliary Surg Nutr. 3(1): 18–34PubMedPubMedCentral
15.
go back to reference Deshpande V, Zen Y, Chan JK, et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181–1192.CrossRefPubMed Deshpande V, Zen Y, Chan JK, et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181–1192.CrossRefPubMed
16.
go back to reference Yata M, Suzuki K, Furuhashi N, Kawakami K, Kawai Y, Naganawa S (2016) Comparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma. Clinical Radiology 71:203–210CrossRefPubMed Yata M, Suzuki K, Furuhashi N, Kawakami K, Kawai Y, Naganawa S (2016) Comparison of the multidetector-row computed tomography findings of IgG4-related sclerosing cholangitis and extrahepatic cholangiocarcinoma. Clinical Radiology 71:203–210CrossRefPubMed
17.
go back to reference Tabata T, Kamisawa T, Hara S, et al (2013) Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma. Gut Liver 7(2):234–238CrossRefPubMedPubMedCentral Tabata T, Kamisawa T, Hara S, et al (2013) Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma. Gut Liver 7(2):234–238CrossRefPubMedPubMedCentral
18.
go back to reference Malaguarnera G, Paladina I, Giordano M, Malaguarnera M, Bertino G, Berretta M (2013) Serum markers of intrahepatic cholangiocarcinoma. Dis Markers 34(4):219.CrossRefPubMedPubMedCentral Malaguarnera G, Paladina I, Giordano M, Malaguarnera M, Bertino G, Berretta M (2013) Serum markers of intrahepatic cholangiocarcinoma. Dis Markers 34(4):219.CrossRefPubMedPubMedCentral
19.
go back to reference Ohara H, Okazaki K, Tsubouchi H et al (2012) Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci. 19(5):536–42CrossRefPubMed Ohara H, Okazaki K, Tsubouchi H et al (2012) Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci. 19(5):536–42CrossRefPubMed
Metadata
Title
Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center
Authors
Jordan Swensson
Temel Tirkes
Mark Tann
Enming Cui
Kumaresan Sandrasegaran
Publication date
01-06-2019
Publisher
Springer US
Published in
Abdominal Radiology / Issue 6/2019
Print ISSN: 2366-004X
Electronic ISSN: 2366-0058
DOI
https://doi.org/10.1007/s00261-019-01944-1

Other articles of this Issue 6/2019

Abdominal Radiology 6/2019 Go to the issue