Roux M, Germain M A
J Chir (Paris). 1980 May;117(5):285-91.
Primary sclerosing cholangitis is a rare affection. Two characteristic cases are described. A total of 80 cases have been reported in the published literature, and lesions are found varying from lacalized to diffuse forms. The affection is often associated with other disorders, especially hemorrhagic ulcerative rectocolitis, suggesting an auto-immune pathogenicity. Treatment is mainly surgical and consists, in the localized forms, of complete excision whenever possible and a by-pass operation. The diffuse forms, which are difficult to treat by surgicall methods, have a poorer prognosis and usually progress towards secondary biliary cirrhosis.
原发性硬化性胆管炎是一种罕见的病症。描述了两个典型病例。已发表的文献中共报道了80例病例，病变表现从局限性到弥漫性不等。这种病症常与其他疾病相关，尤其是出血性溃疡性直肠结肠炎，提示其自身免疫性发病机制。治疗主要是手术治疗，对于局限性病变，尽可能进行完整切除并实施旁路手术。弥漫性病变难以通过手术方法治疗，预后较差，通常会发展为继发性胆汁性肝硬化。
