Localized intrahepatic IgG4-related sclerosing cholangitis (IgG4-SC) as an additional type of IgG4-SC: a systematic analysis of 12 cases

• Published in: Scandinavian journal of gastroenterology p. 1
• Main Authors: Mo, Zhichao, Ramen, Kuvaneshan, Shan, Yunfeng, Zhou, Mengtao, Zheng, Xiangwu, Wu, Xiuling, Yu, Zhengping, Ji, Xiaoke, Zhang, Qiyu, Zeng, Qiqiang
• Format: Journal Article
• Language: English
• Published: England 04.03.2018
• Subjects: classification; IgG4-related sclerosing cholangitis; intrahepatic
• ISSN: 1502-7708
• DOI: 10.1080/00365521.2018.1424233

IgG4-related sclerosing cholangitis (IgG4-SC), a recently defined disease entity, has been classified into four types based on the stricture regions revealed by cholangiography. However, localized intrahepatic IgG4-SC is not included into the classification. This study aimed to analyze and characterize localized intrahepatic IgG4-SC and justify the inclusion of this type into the classification. PubMed and Embase were searched for studies published from March 2001 to June 2017 reporting localized intrahepatic IgG4-SC. Data were obtained and analyzed from the included articles. Twelve cases of localized intrahepatic IgG4-SC were included. All patients were adults with the median age of 73 years (range 46-78), and had a male preponderance (88.9%). The most common clinical presentation was obstructive jaundice (50%), abdominal pain (25%) and absence of symptoms (25%). On imaging and macroscopically, localized intrahepatic IgG4-SC presented with three subtypes, i.e., mass-forming (n = 6, 50%), stricture (n = 5, 41.7%) and periductal infiltrating (n = 1, 8.3%) subtypes. Among the eight cases with diagnoses reported, six patients were misdiagnosed as intrahepatic cholangiocarcinoma; one was diagnosed as hepatic mass and one as IgG4-SC before biopsy or operation. Information on treatment was available on 10 cases; eight underwent surgical resection, one received steroid treatment alone and one underwent endoscopic biliary drainage. No relapse was noted in patients with surgical resection during a period of followed up. The localized intrahepatic IgG4-SC presents with mass-forming, stricture and periductal infiltrating subtypes, and should be recognized as an additional type of IgG4-SC according to the cholangiographic classification or anatomic site.