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World J Gastroenterol. Nov 21, 2013; 19(43): 7661-7670
Published online Nov 21, 2013. doi: 10.3748/wjg.v19.i43.7661
Published online Nov 21, 2013. doi: 10.3748/wjg.v19.i43.7661
Diagnosis of IgG4-related sclerosing cholangitis
Takahiro Nakazawa, Itaru Naitoh, Kazuki Hayashi, Katsuyuki Miyabe, Shuya Simizu, Takashi Joh, Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya 467-8601, Japan
Author contributions: All authors contributed to this work.
Correspondence to: Takahiro Nakazawa, MD, Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan. tnakazaw@med.nagoya-cu.ac.jp
Telephone: +81-52-8538211 Fax: +81-52-8520952
Received: June 20, 2013
Revised: September 17, 2013
Accepted: September 29, 2013
Published online: November 21, 2013
Processing time: 180 Days and 22.5 Hours
Revised: September 17, 2013
Accepted: September 29, 2013
Published online: November 21, 2013
Processing time: 180 Days and 22.5 Hours
Core Tip
Core tip: IgG4-related sclerosing cholangitis (IgG4-SC) has become a third distinct clinical entity of sclerosing cholangitis. The diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma (CC). IgG4-SC responds well to steroid therapy, whereas PSC is only effectively treated with liver transplantation, and CC requires surgical intervention. IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical, serological, morphological, and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis.