Parés Albert
Liver Unit, Hospital Clínic, IDIBAPS, CIBERehd, University of Barcelona, Barcelona, Spain.
Semin Liver Dis. 2017 Feb;37(1):60-72. doi: 10.1055/s-0036-1597929. Epub 2017 Feb 15.
Despite the presumed immunological pathogenesis of primary biliary cholangitis, no clear or even harmful consequences have resulted from treatments designed to modify the immunological condition. Ursodeoxycholic acid (13-16 mg/kg/d) has, however, clear favorable effects that not only improve biochemical cholestasis, but also delay histological progression. Long-term treatment with ursodeoxycholic acid is associated with excellent transplant-free survival in cases showing a biochemical response at 1 year. Data on the effects of obeticholic acid and fibrates are encouraging. Moreover, recent pilot studies evaluating several biological agents targeting immunity such as different monoclonal antibodies and other drugs that modulate cholestasis are under investigation, although with limited results at present.
尽管原发性胆汁性胆管炎存在免疫发病机制的推测,但旨在改变免疫状况的治疗并未产生明确甚至有害的后果。然而,熊去氧胆酸(13 - 16毫克/千克/天)具有明确的有益作用,不仅能改善生化胆汁淤积,还能延缓组织学进展。对于在1年时出现生化反应的病例,长期使用熊去氧胆酸治疗与无移植的良好生存率相关。奥贝胆酸和贝特类药物的效果数据令人鼓舞。此外,目前正在研究评估几种针对免疫的生物制剂(如不同的单克隆抗体和其他调节胆汁淤积的药物)的近期试点研究,尽管目前结果有限。