
IgG4-related cholangitis : case report and literature review
- Author
- Anneleen De Both (UGent) , Hans Van Vlierberghe (UGent) , Anja Geerts (UGent) , Louis Libbrecht (UGent) and Xavier Verhelst (UGent)
- Organization
- Abstract
- Case Presentation : We describe a case of a patient who presents with jaundice, elevated cholestatic liver enzymes, an extreme weight loss and a midcholedochal stricture very suspect for a -cholangiocarcinoma. In the conviction of malignancy, although the absence of anatomopathological prove, the patient underwent a choledochal resection. The anatomopathological specimen revealed no malignancy. In the year following resection, the patient keeps presenting with bile duct strictures and further weight loss. -Ultimately the diagnosis of Ig G4-related cholangitis is withheld. Therapy with corticosteroids is initiated with a spectacular clinical, biochemical and radiographical result. Discussion : IgG4-related cholangitis is the biliary presentation of IgG4-related disease, a recently discovered entity of fibroinflammatory masses which can affect virtually every organ in the body. It is characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis and a presence of >30 IgG4-positive plasma cells per high power field. Main differential -diagnosis contains cholangiocarcinoma and primary sclerosing cholan-gitis. Corticoids are cornerstone of therapy, with azathioprine frequently used as a maintenance in case of relapse. Conclusion : With this case we want to draw the attention to a rather uncommon cause of biliary obstruction, easily mistaken for a cholangiocarcinoma.
- Keywords
- IgG4-related disease, IgG4-related cholangitis, biliary obstruction, IMMUNOGLOBULIN G4-ASSOCIATED CHOLANGITIS, G4-RELATED SCLEROSING CHOLANGITIS, CLINICOPATHOLOGICAL-ENTITY, DISEASE, CHOLANGIOCARCINOMA, PANCREATITIS
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Citation
Please use this url to cite or link to this publication: http://hdl.handle.net/1854/LU-6847834
- MLA
- De Both, Anneleen et al. “IgG4-related Cholangitis : Case Report and Literature Review.” ACTA GASTRO-ENTEROLOGICA BELGICA 78.1 (2015): 62–64. Print.
- APA
- De Both, A., Van Vlierberghe, H., Geerts, A., Libbrecht, L., & Verhelst, X. (2015). IgG4-related cholangitis : case report and literature review. ACTA GASTRO-ENTEROLOGICA BELGICA, 78(1), 62–64.
- Chicago author-date
- De Both, Anneleen, Hans Van Vlierberghe, Anja Geerts, Louis Libbrecht, and Xavier Verhelst. 2015. “IgG4-related Cholangitis : Case Report and Literature Review.” Acta Gastro-enterologica Belgica 78 (1): 62–64.
- Chicago author-date (all authors)
- De Both, Anneleen, Hans Van Vlierberghe, Anja Geerts, Louis Libbrecht, and Xavier Verhelst. 2015. “IgG4-related Cholangitis : Case Report and Literature Review.” Acta Gastro-enterologica Belgica 78 (1): 62–64.
- Vancouver
- 1.De Both A, Van Vlierberghe H, Geerts A, Libbrecht L, Verhelst X. IgG4-related cholangitis : case report and literature review. ACTA GASTRO-ENTEROLOGICA BELGICA. 2015;78(1):62–4.
- IEEE
- [1]A. De Both, H. Van Vlierberghe, A. Geerts, L. Libbrecht, and X. Verhelst, “IgG4-related cholangitis : case report and literature review,” ACTA GASTRO-ENTEROLOGICA BELGICA, vol. 78, no. 1, pp. 62–64, 2015.
@article{6847834, abstract = {Case Presentation : We describe a case of a patient who presents with jaundice, elevated cholestatic liver enzymes, an extreme weight loss and a midcholedochal stricture very suspect for a -cholangiocarcinoma. In the conviction of malignancy, although the absence of anatomopathological prove, the patient underwent a choledochal resection. The anatomopathological specimen revealed no malignancy. In the year following resection, the patient keeps presenting with bile duct strictures and further weight loss. -Ultimately the diagnosis of Ig G4-related cholangitis is withheld. Therapy with corticosteroids is initiated with a spectacular clinical, biochemical and radiographical result. Discussion : IgG4-related cholangitis is the biliary presentation of IgG4-related disease, a recently discovered entity of fibroinflammatory masses which can affect virtually every organ in the body. It is characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis and a presence of >30 IgG4-positive plasma cells per high power field. Main differential -diagnosis contains cholangiocarcinoma and primary sclerosing cholan-gitis. Corticoids are cornerstone of therapy, with azathioprine frequently used as a maintenance in case of relapse. Conclusion : With this case we want to draw the attention to a rather uncommon cause of biliary obstruction, easily mistaken for a cholangiocarcinoma.}, author = {De Both, Anneleen and Van Vlierberghe, Hans and Geerts, Anja and Libbrecht, Louis and Verhelst, Xavier}, issn = {0001-5644}, journal = {ACTA GASTRO-ENTEROLOGICA BELGICA}, keywords = {IgG4-related disease,IgG4-related cholangitis,biliary obstruction,IMMUNOGLOBULIN G4-ASSOCIATED CHOLANGITIS,G4-RELATED SCLEROSING CHOLANGITIS,CLINICOPATHOLOGICAL-ENTITY,DISEASE,CHOLANGIOCARCINOMA,PANCREATITIS}, language = {eng}, number = {1}, pages = {62--64}, title = {IgG4-related cholangitis : case report and literature review}, volume = {78}, year = {2015}, }