臨牀消化器内科 Vol.13 No.2(2)


特集名 原発性硬化性胆管炎(PSC)
題名 PSCの病因をめぐって
発刊年月 1998年 02月
著者 石井 元康 東北大学医学部第三内科
【 要旨 】 原発性硬化性胆管炎(PSC)は,種々の原因で胆管周囲の慢性線維性炎症を呈すると考えられる症候群である.PSCは高頻度に潰瘍性大腸炎(UC)を合併するので,UCと共通の免疫学的機序(大腸粘膜上皮と胆管上皮の共通抗原の存在,抗好中球抗体の存在など)が存在する可能性がある.また,大腸で作られた有毒物質(エンドトキシン,フォルミルペプチド,サイトカインなど)が門脈を介し肝内に達し,胆管炎を惹起する可能性がある.他に虚血なども考えられているが,依然仮説にとどまっている.今後,個々の仮説の可能性を,動物モデルや細胞生物学的手法を用いて検証することが必要である.
Theme Primary Sclerosing Cholangitis
Title Etiology of Primary Sclerosing Cholangitis
Author Motoyasu Ishii Third Department of Internal Medicine, Tohoku University School of Medicine
[ Summary ] Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic liver disease, the etiology of which is supposedly heterogeneous and remains poorly defined. Several hypotheses on the etiology have been proposed to explain the association of PSC with ulcerative colitis, including autoimmunity, inflammation caused by toxic substances produced by enteric flora in the inflamed intestine, and abnormal immunoregulation by cytokines. Furthermore, ischemic vascular damage may also contribute to bile duct damage. Fibrogenesis around the bile ducts follows chronic inflammation, producing a characteristic annular fibrosis around the bile ducts. Several important molecular mechanisms regulating inflammation and fibrogenesis have been revealed by recent immunological and cell biological studies. Although our understanding of PSC etiology has been improved with modern medicine and the availability of animal models, further work is needed to test hypotheses using techniques of immunology and cell biology.
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