Is peripartum cardiomyopathy an organ-specific autoimmune disease?
Is peripartum cardiomyopathy an organ-specific autoimmune disease?
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围产期心肌病是一种器官特异性自身免疫性疾病吗？
DOI：
10.1016/s1568-9972(01)00009-x
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发表时间：
2002
影响因子：
13.6
通讯作者：
Ansari,AftabA
中科院分区：
文献类型：
--
作者：
Sundstrom,JBruce;Fett,JamesD;Carraway,RobertD;Ansari,AftabA
围产期心肌病（PPCM）是一种罕见而严重的心脏病，专门困扰育龄妇女。症状包括在妊娠期间发生的心血管功能不全的快速发作，在没有任何其他心脏病体征或病史的情况下，在妊娠晚期至产后5个月之间的任何时间开始。PPCM的罕见发病率和缺乏任何相关的动物模型限制了对所涉及的致病机制的研究和理解。几组令人信服的数据支持PPCM是自身免疫IDCM的一种形式的观点。然而，PPCM与自身免疫性IDCM的不同之处在于（a）它与独特的自身抗体和自身抗原组相关，（B）它具有相对快速的发作，以及（c）它仅影响孕妇。此外，PPCM的病因取决于妊娠相关因素的相互作用，例如增加的血流动力学应激、血管活性激素和胎儿微嵌合体，其在疾病进展的基本免疫和遗传环境的背景下协同作用。我们的PPCM模型试图代表多种因素，例如妊娠，遗传学，免疫失调和胎儿微嵌合体如何保持复杂的动态平衡，可以共同维持母亲的心血管健康或疾病（图1）。对心脏组织自身抗原的确切性质进行更彻底的研究，可能会导致对自身耐受性破坏机制的鉴定，也可能导致对假定的病原体的鉴定。进一步研究心脏组织自身抗原的确切性质以及控制外周耐受和自身免疫之间平衡的特定因素，例如心脏组织上PD-L1的表达和调节性T细胞的作用，可能有助于阐明PPCM的自身免疫机制。
Peripartum cardiomyopathy (PPCM) is a rare and serious heart disease that exclusively afflicts women during childbearing years. Symptoms include rapid onset of cardiovascular insufficiency occurring during pregnancy, initiated anytime between the third trimester until 5 months post-partum in the absence of any other signs or history of heart disease. The rare incidence of PPCM and the absence of any relevant animal models have limited research and understanding of the pathogenic mechanisms involved. Several compelling sets of data support the view that PPCM is a form of autoimmune IDCM. However, PPCM differs from autoimmune IDCM in that (a) it is associated with unique sets of autoantibodies and autoantigens, (b) it has a relatively rapid onset, and (c) it exclusively affects pregnant women. Furthermore, the etiology of PPCM is dependent on the interaction of pregnancy associated factors, e.g. increased hemodynamic stress, vasoactive hormones and fetal microchimerism, that co-operate in the context of essential immune and genetic environments for disease progression. Our model of PPCM attempts to represent how multiple factors, e.g. pregnancy, genetics, immune dysregulation, and fetal microchimerism are held in a complex dynamic balance that can co-operate towards the maintenance of cardiovascular health or disease in the mother (Fig. 1). A more thorough study of the precise nature of the cardiac tissue autoantigens may lead to the identification of the mechanisms of breakdown of self-tolerance and perhaps also the putative etiologic agent(s). Further studies of the precise nature of the cardiac tissue autoantigens and the specific factors governing the balance between tolerance and autoimmunity in the periphery, e.g. expression of PD-L1 on cardiac tissues and the role of regulatory T cells, may help to elucidate the autoimmune mechanisms of PPCM.