IgG4 Sclerosing Cholangitis – an Inflammation Imitating Tumour of the Pancreas and Biliary Tract

Czech version

Authors: P. Peňázová ¹; T. Andrašina ²; I. Novotný ^1,3; J. Trna ^3–5; B. Hemmelová ¹; L. Kunovsky ^1,4; V. Procházka ¹; Z. Kala ¹
Authors‘ workplace: Chirurgická klinika LF MU a FN Brno ¹; Klinika radiologie a nukleární medicíny LF MU a FN Brno ²; Gastroenterologické oddělení, Masarykův onkologický ústav, Brno ³; Interní gastroenterologická klinika LF MU a FN Brno ⁴; Gastroenterologická ambulance, Nemocnice Boskovice ⁵
Published in: Klin Onkol 2019; 32(2): 143-151
Category: Case Report
doi: https://doi.org/10.14735/amko2019143

Overview

Background:

Immunoglobulin (Ig) G4 associated sclerosing cholangitis is a rare inflammatory disease of the biliary tract. Although it is a very progressive condition, it responds to steroid therapy. IgG4 associated sclerosing cholangitis can mimic pancreatic carcinoma, cholangiocarcinoma, and primary sclerosing cholangitis; therefore, it is very important to obtain a differential diagnosis. IgG4 sclerosing cholangitis is a biliary form of IgG4 related systemic disease, in which afflictions of more organs is afflictions of more organs are common, typically biliary form together with pancreatic one. Nonspecific symptoms are obstructive icterus, fatigue, and weight loss. Atypical imaging of the biliary tree and pancreas can be used to distinguish it from other diseases. Laboratory data show elevation of bilirubin, liver enzymes, IgG4 and total IgG concentrations. Sometimes IgE is also elevated with the eosinophilia, oncomarker CA 19-9 and autoimmune antibody is sometimes detected.

Case:

This article presents a case of IgG4 sclerosing cholangitis and its related findings. The patient was intially referred for suspected pancreatic tumour, the presumed diagnosis was later changed to cholangiocarcinoma type 4 with concurrent autoimmune pancreatitis. Atypical imaging in cholangiography made us suspect IgG4 inflammation and the diagnostic process began.

Conclusion:

The diagnosis of this disease uses so called HISORt criteria. It is a very complex process in which the success of steroid therapy as a final step can be conclusive, as it was in our case. It is essential to exclude a malign neoplastic growth.

The authors declare they have no potential confl icts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers.

Submitted: 5. 12. 2018

Accepted: 10. 1. 2019

Keywords:

IgG4 related systemic disease – immunoglobulin G – IgG4-positive plasma cells – sclerosing cholangitis – cholangiocarcinoma – cancer of pancreas – cholangiography – prednison

Sources

1. Ghazale A, Chari ST, Zhang L et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134 (3): 706–715. doi: 10.1053/j.gastro.2007.12.009.

2. Culver EL, Sadler R, Bateman AC et al. Immunoglobulin E, eosinophils and mast cells in atopic individuals provide novel insights in IgG4-related disease. J Hepatol 2016; 64 (2): S646. doi: 10.1016/S0168-8278 (16) 01206-X.

3. Culver EL, Barnes E. IgG4-related sclerosing cholangitis. Clin Liver Dis 2017; 10 (1): 9–16. doi: 10.1002/ cld.642.

4. Muhi A, Ichikawa T, Motosugi U et al. Mass-form in g autoimmune pancreatitis and pancreatic carcinoma: differential diagnosis on the basis of computed tomography and magnetic resonance cholangiopancreatography, and diffusion-weighted imaging findings. J Magn Reson Imaging 2012; 35 (4): 827–36. doi: 10.1002/jmri.22881.

5. Nishino T, Toki F, Oyama H et al. Biliary tract involvement in autoimmune pancreatitis. Pancreas 2005; 30 (1): 76–82.

6. Kanno A, Masamune A, Okazaki K et al. Nation wide epidemiological survey of autoimmune pancreatitis in Japan in 2011. Pancreas 2015; 44 (4): 535–539. doi: 10.1097/MPA.0000000000000325.

7. Dítě P, Trna J, Kinkor Z et al. Unusual multiorgan immunoglobulin G4 inflammation: autoimmune pancreatitis, mikulicz syndrome and IgG4 mastitis. Gut Liver 2013; 7 (5): 621–624. doi: 10.5009/gnl.2013.7.5.621.

8. Webster GJ, Pereira SP, Chapman RW. Autoimmune pancreatitis/IgG4-associated cholangitis and primary sclerosing cholangitis – overlapping or separate diseases? J Hepatol 2009; 51 (2): 398–402. doi: 10.1016/ j.jhep.2009.04.010.

9. Deshpande V, Zen Y, Chan JK et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012; 25 (9): 1181–1192. doi: 10.1038/modpathol. 2012.72.

10. Bateman AC, Culver EL. IgG4-related disease – experience of 100 consecutive cases from a specialist centre. Histopathology 2017; 70 (5): 798–813. doi: 10.1111/his.13136.

11. Trna J, Kala Z (eds). Klinická pankreatologie. 1. vyd. Praha: Mladá fronta 2016.

12. Hur BY, Lee JM, Lee JE et al. Magnetic resonance imaging findings of the mass-forming type of autoimmune pancreatitis: comparison with pancreatic adenocarcinoma. J Magn Reson Imaging 2012; 36 (1): 188–197. doi: 10.1002/jmri.23609.

13. Ohara H, Okazaki K, Tsubouchi H et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 2012; 19 (5): 536–542. doi: 10.1007/s00534-012-0521-y.

14. Mendes FD, Jorgensen R, Keach J et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006; 101 (9): 2070–2075. doi: 10.1111/j.1572-0241.2006.00772.x.

15. Dítě P, Novotný I, Lata J et al. Autoimunitní pankreatitida a IgG4 pozitivní sklerotizující cholangitida. Vnitř Lék 2011; 57 (3): 254–257.