TRIALS OF THERAPIES FOR PRIMARY BILLIARY CIRRHOSIS

原发性二元性肝硬化是一种病因不明的疾病 以缓慢进行性肝内胆汁淤积为特征， 非化脓性，肝内小胆管破坏。因为有些 其他自身免疫性疾病似乎对某些 免疫抑制剂，选定的免疫抑制药物的试验是 在有症状的PBC患者中进行。以随机 苯丁酸氮芥治疗的对照试验， 与血清胆红素升高速率的降低相关， 血清IgM水平升高恢复正常并改善 肝活检中炎性细胞浸润。这些发现 促使人们寻找更安全、更有效的免疫抑制药物， 比苯丁酸氮芥致癌性低。10例PBC患者 已进入甲氨蝶呤治疗的开放试验， 药物很好。

Primary binary cirrhosis (PBC) is a disease of unknown etiology characterized by slowly progressive intrahepatic cholestasis due to non-suppurative, destruction of small intrahepatic bile ducts. Because some other autoimmune diseases appear to respond favorably to certain immunosuppressive agents, trials of selected immunosuppressive drugs are being undertaken in patients with symptomatic PBC. In a randomized controlled trial of chlorambucil therapy, treatment with the drug was associated with a decrease in the rate of increase of serum bilirubin, normalization of elevated serum IgM levels and an improvement in inflammatory cell infiltration in liver biopsies. These findings have prompted a search for safer and more effective immunosuppressive drugs with less carcinogenic potential than chlorambucol. Ten patients with PBC have been entered into an open trial of methotrexate therapy and are tolerating the drug well.