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

  • Hepatobiliary
  • Published:
Abdominal Radiology Aims and scope Submit manuscript

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.

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References

  1. 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 54:535–551

    Article  PubMed  Google Scholar 

  2. Vlachou P, Khalili K, Jang HJ, Fischer S, Hirschfield GM, Kim TK (2011) IgG4-related Sclerosing Disease: Autoimmune Pancreatitis and Extrapancreatic Manifestations. Radiographics 31:1379–1402

    Article  PubMed  Google Scholar 

  3. 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 35:240–254

    Article  Google Scholar 

  4. Itoh S, Nagasaka T, Suzuki K, Ota T, Naganawa S (2009) Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings. Clinical Radiology 64:1104–1114

    Article  CAS  PubMed  Google Scholar 

  5. 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): 152–157

    Article  PubMed  Google Scholar 

  6. 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 54:601–607

    Article  PubMed  Google Scholar 

  7. 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 20:190–196

    Article  PubMed  Google Scholar 

  8. 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 25:1831–1837

    Article  PubMed  Google Scholar 

  9. 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 22:1287–1295

    Article  CAS  PubMed  Google Scholar 

  10. Sivakumaran Y, Le Page PA, Becerril-Martinez G et al (2014) IgG4-related sclerosing cholangitis: the cholangiocarcinoma mimic. ANZ J Surg 84:486–487

    Article  PubMed  Google Scholar 

  11. Ghazale A, Chari S, Smyrk T, Takahashi N, Levy M (2008) Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy. Gastroenterology 134:706–715

    Article  PubMed  Google Scholar 

  12. Khosroshahi A, Stone JH (2011) A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol 23(1):57–66

    Article  CAS  PubMed  Google Scholar 

  13. Culver EL, Barnes E (2017) IgG4-related sclerosing cholangitis. Clinical Liver Disease 10(1): 9–16

    Article  PubMed  PubMed Central  Google Scholar 

  14. Soares KC, Kamel I, Cosgrove DP, Herman JM, Pawlik TM (2014) Hilar cholangiocarcinoma: diagnosis, treatment options, and management. Hepatobiliary Surg Nutr. 3(1): 18–34

    PubMed  PubMed Central  Google Scholar 

  15. Deshpande V, Zen Y, Chan JK, et al (2012) Consensus statement on the pathology of IgG4-related disease. Mod Pathol 25:1181–1192.

    Article  PubMed  Google Scholar 

  16. 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 71:203–210

    Article  CAS  PubMed  Google Scholar 

  17. Tabata T, Kamisawa T, Hara S, et al (2013) Differentiating immunoglobulin g4-related sclerosing cholangitis from hilar cholangiocarcinoma. Gut Liver 7(2):234–238

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Malaguarnera G, Paladina I, Giordano M, Malaguarnera M, Bertino G, Berretta M (2013) Serum markers of intrahepatic cholangiocarcinoma. Dis Markers 34(4):219.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Ohara H, Okazaki K, Tsubouchi H et al (2012) Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci. 19(5):536–42

    Article  PubMed  Google Scholar 

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Funding

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Authors and Affiliations

  1. Department of Radiology and Imaging Sciences, Indiana University School of Medicine, 550 N University Blvd Room 0663, Indianapolis, IN, 46202, USA

    Jordan Swensson, Temel Tirkes & Mark Tann

  2. Department of Radiology, Jiangmen Central Hospital, 23 Haibang Street, Pengjiang District, Jiangmen, 529030, Guangdong, People’s Republic of China

    Enming Cui

  3. Department of Radiology, Mayo Clinic, Scottsdale, AZ, USA

    Kumaresan Sandrasegaran

Authors
  1. Jordan Swensson
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  2. Temel Tirkes
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  3. Mark Tann
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  4. Enming Cui
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  5. Kumaresan Sandrasegaran
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Correspondence to Jordan Swensson.

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Conflict of interest

Dr. Temel Tirkes was supported by National Cancer Institute and National Institute of Diabetes and Digestive and Kidney Diseases of the National Institutes of Health under Award Numbers 1R01DK116963-01A1 and U01DK108323 (Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer). The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

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Swensson, J., Tirkes, T., Tann, M. et al. Differentiating IgG4-related sclerosing cholangiopathy from cholangiocarcinoma using CT and MRI: experience from a tertiary referring center. Abdom Radiol 44, 2111–2115 (2019). https://doi.org/10.1007/s00261-019-01944-1

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  • DOI: https://doi.org/10.1007/s00261-019-01944-1

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