臨牀消化器内科 Vol.25 No.9(7)


特集名 自己免疫性膵炎
題名 自己免疫性膵炎に伴う硬化性胆管炎(IgG4 関連硬化性胆管炎)
発刊年月 2010年 08月
著者 内藤 格 名古屋市立大学医学部消化器・代謝内科学
著者 大原 弘隆 名古屋市立大学病院総合内科
著者 中沢 貴宏 名古屋市立大学医学部消化器・代謝内科学
著者 安藤 朝章 名古屋市立大学医学部消化器・代謝内科学
著者 林 香月 名古屋市立大学医学部消化器・代謝内科学
著者 城 卓志 名古屋市立大学医学部消化器・代謝内科学
【 要旨 】 自己免疫性膵炎(AIP)は高頻度に硬化性胆管炎を合併し,IgG4関連硬化性胆管炎(IgG4-SC)と称される.IgG4-SCにはステロイドが著効するため,治療法,予後が異なる原発性硬化性胆管炎(PSC)や胆管癌との鑑別が重要である.診断にはAIP合併の有無,血清IgG4値,全身合併症の有無などを参考にすることが必要である.IgG4-SCとPSCは胆管像を詳細に検討することでおおよそ鑑別が可能であり,さらにPSCには大腸内視鏡における右側結腸に病勢が強い炎症性腸疾患の合併が参考になる.IgG4-SCと胆管癌の鑑別には胆管腔内超音波検査における胆管狭窄部の所見と非狭窄部における胆管壁肥厚が有用である.
Theme Autoimmune Pancreatitis
Title Sclerosing Cholangitis Associatedwith Autoimmune Pancreatitis (IgG4-relatedSclerosing Cholangitis)
Author Itaru Naitoh Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences
Author Hirotaka Ohara Division of General Medicine, Nagoya City University Hospital
Author Takahiro Nakazawa Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences
Author Tomoaki Ando Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences
Author Kazuki Hayashi Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences
Author Takashi Joh Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences
[ Summary ] Autoimmune pancreatitis (AIP) is frequently associated with sclerosing cholangitis termed IgG4-related sclerosing cholangitis (IgG4-SC). Primary sclerosing cholangitis (PSC) and cholangiocarcinoma (CC) are important diseases with differing diagnoses because these diseases are different from IgG4-SC in both means of treatment and prognosis. IgG4-SC usually responds well to steroid therapy. In contrast,liver transplantation is the only effective therapy for PSC and surgical procedures are required for chlangiocarcinoma. High serum IgG4 values,coexistence with AIP and other organ involvement are useful factors for diagnosing IgG4-SC. The association with right-dominant inflammatory bowel syndrome suggests PSC. Cholangiograms are useful for discriminating IgG4-SC from PSC. Band-like strictures,beaded appearance and pruned tree appearance are characteristicly seen in cholangiograms for PSC. Segmental strictures,long strictures with prestenotic dilation and lower common bile duct strictures are characteristic findings associated with IgG4-SC. Intraductal ultrasonograpy(IDUS)findings in the regions of strictures and non-strictured regions as observed in cholangiograms are useful to discriminate IgG4-SC from CC. The most characteristic IDUS finding of IgG4-SC is wall thickening in non-strictured regions,as observed in cholangiograms which discriminate it from CC. Wall thickness spreads from the intrapancreatic bile duct to the upper bile duct continuously in most IgG4-SC cases.
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