臨牀消化器内科 Vol.13 No.2(5)


特集名 原発性硬化性胆管炎(PSC)
題名 PSCの臨床病態
発刊年月 1998年 02月
著者 東 克謙 名古屋市立大学医学部第一内科
【 要旨 】 原発性硬化性胆管炎(PSC)は肝内・外胆管のびまん性硬化性病変により,慢性胆汁うっ滞を呈する比較的まれな疾患であるが,最近,本疾患への関心の高まりとともに症例数が増加している.PSCの診断基準を満たすPSC症例には,経過によって病変が進展していく定型例と,一過性の閉塞性黄疸を呈し胆道ドレナージ,薬物治療により軽快する非定型例の2群のあることが示された.定型例においては発症年齢が明らかに若年であり,両者では臨床病態が異なっているものと考えられる.若年者発症例はほとんど定型例であり,病変は進行性であるが,高齢者のPSC症例では非定型例で予後が良好な症例もみられ,種々の病態が考えられる.
Theme Primary Sclerosing Cholangitis
Title Clinical Features of Primary Sclerosing Cholangitis
Author Katsuyoshi Higashi First Department of Internal Medicine, Nagoya City University Medical School
[ Summary ] Primary sclerosing cholangitis (PSC) is an uncommon disease characterized by chronic cholestasis caused by diffuse inflammation and fibrosis that involves the entire biliary tract. In Japan, patients with PSC are clinically classified into the two groups, typical and non-typical cases. The disease is progressive in the typical cases. On the other hand, the cholangiographic findings and liver function tests improve with treatment (biliary drainage and/or drug administration) in the non-typical cases which show the transient obstructive jaundice. Ages of onset in the typical cases were significantly younger than in the non-typical cases. Clinical features appear to differ between these two groups. Most younger patients with PSC are typical cases and the disease is progressive. However, patients with a favorable prognosis are observed among the elderly. It is likely that there are several clinical features in these patients.
戻る