PBC是一种进展性的肝脏自身免疫性疾病，病原学尚不清楚，几乎所有PBC均发生在女性中，目前熊去氧胆酸是唯一被食品药品管理局批准的用于PBC病人的药物。尽管PBC确切的发病机制尚不清除，有假设认为许多细胞群，包括B细胞，参与了持续的炎症过程，因此对于把消耗B细胞作为这一疾病的潜在治疗靶点，我们并不惊讶。利妥昔单抗是嵌合型抗CD20单克隆抗体，已被批准用于淋巴瘤和自身免疫性疾病如类风湿性关节炎的治疗。但在PBC的治疗中是否有效尚不知道。最近，利妥昔单抗引起的B细胞消耗显著减少了产生AMA的B细胞，降低了AMA滴度，血浆免疫球蛋白水平以及血清碱性磷酸酶水平，并被治疗病人很好的耐受，无严重不良反应。这一观察为那些对熊去氧胆酸治疗反应不佳的PBC病人提供了治疗的新选择。



　　PBC is a progressive autoimmune liver disease of unknown etiology that affects almost exclusively women. UDCA is currently the only approved drug by Food and Drug Administration for patients with PBC. Although the precise pathogenesis of PBC remains unclear, it has been postulated that many cell populations, including B cells, are involved in the ongoing inflammatory process, which implicates, not surprisingly, a potential therapeutic target of depleting B cell to treat this disorder. Rituximab is a chimeric anti-CD20 monoclonal antibody that has been approved for the treatment of lymphoma and some autoimmune diseases such as rheumatoid arthritis. Whether it is effective in the treatment of PBC has not been evaluated. Recently, Tsuda etal demonstrated that B cell depletion with rituximab significantly reduced the number of AMA-producing B cells, AMA titers, the plasma levels of immunoglobulins as well as serum ALP, and it was well tolerated by all the treated patients with no serious adverse events. This observation provides a novel treatment option for the patients with PBC who have incomplete response to UDCA.

点击下载 B细胞消耗在原发性胆汁性肝硬化（PBC）治疗中的利弊