JCI Insight. 2017 Mar 9;2(5):e92021. doi: 10.1172/jci.insight.92021.
Despite the rising incidence of autoimmunity, therapeutic options for patients with autoimmune disease still rely on decades-old immunosuppressive strategies that risk severe and potentially fatal complications. Thus, novel therapeutic approaches for autoimmune diseases are greatly needed in order to minimize treatment-related toxicity. Such strategies would ideally target only the autoreactive immune components to preserve beneficial immunity. Here, we review how several decades of basic, translational, and clinical research on the immunology of pemphigus vulgaris (PV), an autoantibody-mediated skin disease, have enabled the development of targeted immunotherapeutic strategies. We discuss research to elucidate the pathophysiology of PV and how the knowledge afforded by these studies has led to the preclinical and clinical testing of targeted approaches to neutralize autoantibodies, to induce antigen-specific tolerance, and to specifically eliminate autoreactive B cells in PV.
尽管自身免疫的发病率不断上升，但对于自身免疫性疾病患者的治疗选择仍然依赖于数十年前的免疫抑制策略，这些策略存在严重且潜在致命的并发症风险。因此，非常需要针对自身免疫性疾病的新型治疗方法，以便最大程度地减少治疗相关的毒性。这些策略理想情况下应仅针对自身反应性免疫成分，以保留有益的免疫。在这里，我们回顾了几十年来对寻常型天疱疮（PV）的免疫学的基础、转化和临床研究，这些研究使靶向免疫治疗策略得以发展。我们讨论了阐明 PV 病理生理学的研究，以及这些研究提供的知识如何导致针对中和自身抗体、诱导抗原特异性耐受和特异性消除 PV 中自身反应性 B 细胞的靶向方法的临床前和临床测试。
