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Diagnostic procedures for IgG4‐related sclerosing cholangitis
Author(s) -
Nakazawa Takahiro,
Ando Tomoaki,
Hayashi Kazuki,
Naitoh Itaru,
Ohara Hirotaka,
Joh Takashi
Publication year - 2011
Publication title -
journal of hepato‐biliary‐pancreatic sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.63
H-Index - 60
eISSN - 1868-6982
pISSN - 1868-6974
DOI - 10.1007/s00534-010-0320-2
Subject(s) - autoimmune pancreatitis , medicine , primary sclerosing cholangitis , liver transplantation , stenosis , gastroenterology , autoimmune hepatitis , biopsy , bile duct , cholangiography , pancreatitis , inflammatory bowel disease , radiology , pathology , hepatitis , disease , transplantation
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.