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Immunoglobulin G4-associated sclerosing cholangitis in a patient with Crohn’s disease


Authors: J. Drahoš;  P. Vyhnálek
Authors‘ workplace: Interní oddělení, Pardubická nemocnice, Nemocnice Pardubického kraje, a. s.
Published in: Gastroent Hepatol 2017; 71(1): 49-52
Category: Hepatology: Case Report
doi: https://doi.org/10.14735/amgh2016csgh.info14

Overview

The authors present the case of a 56-year-old patient treated for Crohn’s disease and primary sclerosing cholangitis (PSC). Histologic examination of a liver biopsy, which was performed to evaluate the advancement of liver disease, alerted us to the fact that the patient might have immunoglobulin G4-associated sclerosing cholangitis (IASC). We did not initially consider this disease because the patient lacked some of its characteristic features. He had not suffered from autoimmune pancreatitis and the development of liver disease was slow, with only a slow increase in serum concentrations of cholestatic enzymes. The onset of IASC is usually sudden with obstructive icterus. IASC usually presents in older patients, and its isolated occurrence without autoimmune pancreatitis is described in only 2% of patients. The coincidence of inflammatory bowel disease is also unusual for IASC. A high serum concentration of IgG4 is not sufficient to establish a diagnosis because it can be observed in patients with cholangiocellular cancer or PSC. IASC diagnosis is based on a combination of histological, imaging, biochemical, and clinical findings. However, there are no universally accepted diagnostic criteria. Crucial in our case was good clinical and laboratory response to the administration of corticosteroids, which is one of the essential features of imunoglobulin G4 associated diseases.

Key words:
immunoglobulin G – sclerosing cholangitis – Crohn’s disease

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

The Editorial Board declares that the manuscript met the ICMJE „uniform requirements“ for biomedical papers.

Submitted:
22. 6. 2016

 Accepted:
9. 8. 2016


Sources

1. Nakazawa T, Naitoh I, Hayashi K et al. Dia­gnosis of IgG4-related scleros­ing cholangitis. World J Gastroenterol 2013; 19(43): 7661– 7670. doi: 10.3748/ wjg.v19.i43.7661.

2. Dítě P, Novotný I, Lata J et al. Autoimunitní pankreatitida a IgG pozitivní sklerotizující cholangitida. Vnitr Lek 2011; 57(3): 254– 257.

3. Hubers LM, Beuers U. How to dia­gnose im­munoglobulin g4-as­sociated cholangitis: the jack-of-al­l-trades in the biliary tract. Viszeralmedizin 2015; 31(3): 185– 188. doi: 10.1159/ 000431028.

4. Inoue D, Yoshida K, Yoneda N et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine 2015; 94(15): e680. doi: 10.1097/ MD.0000000000000680.

5. 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.

6. Zen Y, Nakanuma Y. IgG4 Cholangiopathy. Int J Hepatol 2012; 2012: 472376. doi: 10.1155/ 2012/ 472376.

7. Zen Y, Kawakami H, Kim JH. IgG4-related scleros­ing cholangitis: all we need to know. J Gastroenterol 2016; 51(4): 295– 312. doi: 10.1007/ s00535-016-1163-7.

8. Oseini AM, Chaiteerakij R, Shire AM et al. The utility of serum im­munoglobulin G4 in distinguish­ing im­munoglobulin G4--as­sociated cholangitis from cholangiocarcinoma. Hepatology 2011; 54(3): 940– 948. doi: 10.1002/ hep.24487.

9. 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.

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

Labels
Paediatric gastroenterology Gastroenterology and hepatology Surgery

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Gastroenterology and Hepatology

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2017 Issue 1

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