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Journal of Hepato-Biliary-Pancreatic Sciences

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01-03-2011 | Review article

Diagnostic procedures for IgG4-related sclerosing cholangitis

Authors: Takahiro Nakazawa, Tomoaki Ando, Kazuki Hayashi, Itaru Naitoh, Hirotaka Ohara, Takashi Joh

Published in: Journal of Hepato-Biliary-Pancreatic Sciences | Issue 2/2011

Abstract

Background/purpose

IgG4-related sclerosing cholangitis (IgG4-SC) is one of several diseases associated with autoimmune pancreatitis (AIP). However, diffuse cholangraphic abnormalities seen in association with AIP may resemble those seen in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma. IgG4-SC responds well to steroid therapy, whereas in contrast, liver transplantation is the only effective therapy for PSC, and surgical intervention is also needed for cholangiocarcinoma. The aim of this review was to establish the diagnostic procedures for IgG4-SC.

Methods

A literature search was conducted, covering English-language articles dealing with IgG4-SC published between 1991 and March 2010. As clinical data on IgG4-SC are limited, the author also took into consideration his own clinical experience with the treatment of IgG4-SC over a period of more than 19 years.

Results

When intrapancreatic stenosis is detected, pancreatic cancer should be ruled out. If multiple intrahepatic stenosis is evident, PSC should be discriminated on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining. An association with inflammatory bowel disease (IBD) is suggestive of PSC. If stenosis is demonstrated in the hepatic hilar region, cholangiocarcinoma should be discriminated by US, EUS, IDUS, and bile duct biopsy.

Conclusion

For diagnosis of IgG4-SC, coexistence of AIP is the most useful finding. However, the most important consideration for clinicians is to be aware of IgG4-SC when encountering patients with obstructive jaundice.

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Title: Diagnostic procedures for IgG4-related sclerosing cholangitis
Authors: Takahiro Nakazawa
Tomoaki Ando
Kazuki Hayashi
Itaru Naitoh
Hirotaka Ohara
Takashi Joh
Publication date: 01-03-2011
Publisher: Springer Japan
Published in: Journal of Hepato-Biliary-Pancreatic Sciences / Issue 2/2011
Print ISSN: 1868-6974
Electronic ISSN: 1868-6982
DOI: https://doi.org/10.1007/s00534-010-0320-2

At a glance: The STEP trials

Springer Medicine

Diagnostic procedures for IgG4-related sclerosing cholangitis

Abstract

Background/purpose

Methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Background/purpose

Methods

Results

Conclusion

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