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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
Diagnosis of IgG4-related sclerosing cholangitis
Takahiro Nakazawa, Itaru Naitoh, Kazuki Hayashi, Katsuyuki Miyabe, Shuya Simizu, Takashi Joh
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
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.