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Since <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content> shares a number of clinical, biochemical, and imaging features with cholangiocarcinoma (<jats:styled-content style=\"fixed-case\">CCA<\/jats:styled-content>), it is often misdiagnosed as <jats:styled-content style=\"fixed-case\">CCA<\/jats:styled-content>, and unnecessary surgery was performed. In this compact review, we clarify the disease of <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content>, summarize criteria for diagnosis of <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content>, discuss the role of <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content> 19\u20109, and provide key information to differentiate diagnosis of <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content> from <jats:styled-content style=\"fixed-case\">CCA<\/jats:styled-content>. <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content> should be highly suspected in unexplained biliary stricture associated with increased IgG4 (in serum especially in bile) and other organ involvement (kidney, retroperitoneum etc. especially pancreas in which there are abundant <jats:styled-content style=\"fixed-case\">IgG<\/jats:styled-content>4\u2010positive plasmocytes infiltration). Correct diagnosis of <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content> will avoid unnecessary surgery because <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content> responds well to steroid therapy. In a word, increased IgG4 levels, other organ involvement and response to steroids are keys to distinguishing <jats:styled-content style=\"fixed-case\">IAC<\/jats:styled-content> from <jats:styled-content style=\"fixed-case\">CCA<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/j.1440-1746.2012.07287.x","type":"journal-article","created":{"date-parts":[[2012,10,4]],"date-time":"2012-10-04T11:39:05Z","timestamp":1349350745000},"page":"1783-1789","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Autoimmune cholangitis and cholangiocarcinoma"],"prefix":"10.1111","volume":"27","author":[{"given":"Jun","family":"Li","sequence":"first","affiliation":[{"name":"Department of Hepatobiliary Surgery The First Affiliated Hospital of Guangzhou Medical College  Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chaochen","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Hepatobiliary Surgery The First Affiliated Hospital of Guangzhou Medical College  Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuechun","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine The First Affiliated Hospital of Guangzhou Medical College  Guangzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,11,20]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00535-003-1175-y"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2007.12.009"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0016-5107(05)00561-4"},{"key":"e_1_2_8_5_1","first-page":"432","article-title":"IgG4\u2010related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis. 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