Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies.
Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies.
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DOI：
10.3389/fimmu.2021.773352
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发表时间：
2021
影响因子：
7.3
通讯作者：
Coutant F
中科院分区：
文献类型：
--
作者：
Nombel A;Fabien N;Coutant F
抗mda5皮肌炎是一种罕见的全身性自身免疫性疾病，历史上在日本有临床淀粉样皮肌炎和危及生命的快速进行性肺间质性疾病的患者中有描述。随后，该疾病的完整临床谱被皮肤、关节和血管表现所丰富。根据这些症状的优势，现在定义了三种不同的临床表型和不同的预后。迄今为止，这三种实体唯一已知的分子成分是靶向MDA5的特异性抗体，MDA5是抗病毒宿主免疫反应所必需的细胞质蛋白。已经出现了几种检测这些抗体的生物工具，每种工具都有缺点和局限性。然而，这种疾病的高度特异性血清学标记物的鉴定提出了其在发病机制中的作用的问题。尽管目前关于该病发病机制的知识仍处于初级阶段，但有几条证据支持干扰素介导的血管病变在皮肤和肺部病变的发展中发挥核心作用，以及抗mda5抗体可能参与的致病作用。在这里，我们回顾了支持这些假设的临床和生物学证据，并讨论了新兴疗法的贡献，这些疗法揭示了该疾病的发病机制。
Anti-MDA5 dermatomyositis is a rare systemic autoimmune disease, historically described in Japanese patients with clinically amyopathic dermatomyositis and life-threatening rapidly progressive interstitial lung disease. Subsequently, the complete clinical spectrum of the disease was enriched by skin, articular and vascular manifestations. Depending on the predominance of these symptoms, three distinct clinical phenotypes with different prognosis are now defined. To date, the only known molecular component shared by the three entities are specific antibodies targeting MDA5, a cytosolic protein essential for antiviral host immune responses. Several biological tools have emerged to detect these antibodies, with drawbacks and limitations for each of them. However, the identification of this highly specific serological marker of the disease raises the question of its role in the pathogenesis. Although current knowledge on the pathogenic mechanisms that take place in the disease are still in their enfancy, several lines of evidence support a central role of interferon-mediated vasculopathy in the development of skin and lung lesions, as well as a possible pathogenic involvement of anti-MDA5 antibodies. Here, we review the clinical and biological evidences in favor of these hypothesis, and we discuss the contribution of emerging therapies that shed some light on the pathogenesis of the disease.
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DOI：
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发表时间：
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期刊：
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