Foster Mary H
Department of Medicine, Duke University Medical Center, Durham, NC, USA; Durham VA Medical Center, Durham, NC, USA.
Matrix Biol. 2017 Jan;57-58:149-168. doi: 10.1016/j.matbio.2016.07.008. Epub 2016 Aug 2.
Basement membrane components are targets of autoimmune attack in diverse diseases that destroy kidneys, lungs, skin, mucous membranes, joints, and other organs in man. Epitopes on collagen and laminin, in particular, are targeted by autoantibodies and T cells in anti-glomerular basement membrane glomerulonephritis, Goodpasture's disease, rheumatoid arthritis, post-lung transplant bronchiolitis obliterans syndrome, and multiple autoimmune dermatoses. This review examines major diseases linked to basement membrane autoreactivity, with a focus on investigations in patients and animal models that advance our understanding of disease pathogenesis. Autoimmunity to glomerular basement membrane type IV is discussed in depth as a prototypic organ-specific autoimmune disease yielding novel insights into the complexity of anti-basement membrane immunity and the roles of genetic and environmental susceptibility.
基底膜成分是多种疾病自身免疫攻击的靶点,这些疾病会破坏人类的肾脏、肺、皮肤、黏膜、关节和其他器官。特别是,在抗肾小球基底膜肾小球肾炎、肺出血肾炎综合征、类风湿性关节炎、肺移植后闭塞性细支气管炎综合征和多种自身免疫性皮肤病中,胶原蛋白和层粘连蛋白上的表位会受到自身抗体和T细胞的攻击。本综述探讨了与基底膜自身反应性相关的主要疾病,重点关注在患者和动物模型中的研究,这些研究有助于我们理解疾病的发病机制。作为一种典型的器官特异性自身免疫疾病,对IV型肾小球基底膜的自身免疫进行了深入讨论,它为抗基底膜免疫的复杂性以及遗传和环境易感性的作用提供了新的见解。