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Journal of Hepato-Biliary-Pancreatic Sciences

Published in:

01-09-2012 | Guideline

Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012

Authors: Hirotaka Ohara, Kazuichi Okazaki, Hirohito Tsubouchi, Kazuo Inui, Shigeyuki Kawa, Terumi Kamisawa, Susumu Tazuma, Kazushige Uchida, Kenji Hirano, Hitoshi Yoshida, Takayoshi Nishino, Shigeru B. H. Ko, Nobumasa Mizuno, Hideaki Hamano, Atsushi Kanno, Kenji Notohara, Osamu Hasebe, Takahiro Nakazawa, Yasuni Nakanuma, Hajime Takikawa

Published in: Journal of Hepato-Biliary-Pancreatic Sciences | Issue 5/2012

Abstract

Background

IgG4-sclerosing cholangitis (IgG4-SC) patients have an increased level of serum IgG4, dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall, and a good response to steroid therapy. However, it is not easy to distinguish IgG4-SC from primary sclerosing cholangitis, pancreatic cancer, and cholangiocarcinoma on the basis of cholangiographic findings alone because various cholangiographic features of IgG4-SC are similar to those of the above progressive or malignant diseases.

Methods

The Research Committee of IgG4-related Diseases and the Research Committee of Intractable Diseases of Liver and Biliary Tract in association with the Ministry of Health, Labor and Welfare, Japan and the Japan Biliary Association have set up a working group consisting of researchers specializing in IgG4-SC, and established the new clinical diagnostic criteria of IgG4-SC 2012.

Results

The diagnosis of IgG4-SC is based on the combination of the following 4 criteria: (1) characteristic biliary imaging findings, (2) elevation of serum IgG4 concentrations, (3) the coexistence of IgG4-related diseases except those of the biliary tract, and (4) characteristic histopathological features. Furthermore, the effectiveness of steroid therapy is an optional extra diagnostic criterion to confirm accurate diagnosis of IgG4-SC.

Conclusion

These diagnostic criteria for IgG4-SC are useful in practice for general physicians and other nonspecialists.

Available only for authorised users

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Title: Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012
Authors: Hirotaka Ohara
Kazuichi Okazaki
Hirohito Tsubouchi
Kazuo Inui
Shigeyuki Kawa
Terumi Kamisawa
Susumu Tazuma
Kazushige Uchida
Kenji Hirano
Hitoshi Yoshida
Takayoshi Nishino
Shigeru B. H. Ko
Nobumasa Mizuno
Hideaki Hamano
Atsushi Kanno
Kenji Notohara
Osamu Hasebe
Takahiro Nakazawa
Yasuni Nakanuma
Hajime Takikawa
Publication date: 01-09-2012
Publisher: Springer Japan
Published in: Journal of Hepato-Biliary-Pancreatic Sciences / Issue 5/2012
Print ISSN: 1868-6974
Electronic ISSN: 1868-6982
DOI: https://doi.org/10.1007/s00534-012-0521-y

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012

Abstract

Background

Methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusion

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