Among the inflammatory myopathies, anti-tRNA-synthetase syndrome (ASyS) is a severe autoimmune condition characterized by extramuscular involvement, affecting especially the lungs. ASyS specific serological markers are anti-tRNA-synthetase autoantibodies, among which anti-histidyl-tRNA-synthetase is the most common. In the past decades, ASyS has been distinguished by unique histological features attributed to a specific pathogenesis. Research has highlighted the role of environmental factors and infections as possible triggers. Tissue modifications of histidyl-tRNA-synthetase (HisRS) expression might be responsible for the recruitment and activation of both innate and adaptive immune cells. HisRS not only acts through antigenic properties, but also through many others, including chemoattraction, innate pathway activation, and cytokine-like functions. Favored by a certain genetic background, this whole activation of immunity results in widespread and specific tissue damage and finally leads to visible heterogeneous symptoms characterizing the disease state. Understanding the pathogenesis of ASyS is essential to improving patient care by identifying biomarkers and designing new therapeutic strategies accordingly. Therefore, this review details the recent hypotheses concerning the dynamic of ASyS pathogenesis with the aim of enlightening the development of new therapeutic axes in the future.

中文翻译：

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抗合成酶综合症的免疫发病机制。

在炎性肌病中，抗tRNA合成酶综合征（ASyS）是一种严重的自身免疫病，其特征是肌肉外受累，特别是影响肺部。ASyS特异性血清标志物是抗tRNA合成酶自身抗体，其中最常见的是抗组氨酸tRNA合成酶。在过去的几十年中，ASyS的独特组织学特征归因于特定的发病机制。研究强调了环境因素和感染可能是触发因素的作用。组氨酸-tRNA合成酶（HisRS）表达的组织修饰可能负责先天和适应性免疫细胞的募集和激活。HisRS不仅通过抗原特性起作用，而且还通过许多其他特性起作用，包括化学引诱，先天途径激活，和细胞因子样功能。在一定的遗传背景的帮助下，免疫力的整个激活导致广泛而特定的组织损伤，并最终导致表征疾病状态的可见异质症状。通过识别生物标志物并相应地设计新的治疗策略，了解ASyS的发病机理对于改善患者护理至关重要。因此，本综述详细阐述了有关ASyS发病机制动力学的最新假说，目的是启发未来新治疗轴的开发。通过识别生物标志物并相应地设计新的治疗策略，了解ASyS的发病机理对于改善患者护理至关重要。因此，本综述详细阐述了有关ASyS发病机制动力学的最新假说，目的是启发未来新治疗轴的开发。通过识别生物标志物并相应地设计新的治疗策略，了解ASyS的发病机理对于改善患者护理至关重要。因此，本综述详细阐述了有关ASyS发病机制动力学的最新假说，目的是启发未来新治疗轴的开发。