Lung transplantation is a life-saving therapy for several end-stage lung diseases. However, lung allografts suffer from the lowest survival rate predominantly due to rejection. The pathogenesis of alloimmunity and its role in allograft rejection has been extensively studied and multiple approaches have been described to induce tolerance. However, in the context of lung transplantation, dysregulation of mechanisms, which maintain tolerance against self-antigens, can lead to lung-restricted autoimmunity, which has been recently identified to drive the immunopathogenesis of allograft rejection. Indeed, both preexisting as well as de novo lung-restricted autoimmunity can play a major role in the development of lung allograft rejection. The three most widely studied lung-restricted self-antigens include collagen type I, collagen type V, and k-alpha 1 tubulin. In this review, we discuss the role of lung-restricted autoimmunity in the development of both early as well as late lung allograft rejection and recent literature providing insight into the development of lung-restricted autoimmunity through the dysfunction of immune mechanisms which maintain peripheral tolerance.

中文翻译：

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肺损伤和调节性T细胞的丢失引发了肺受限的自身免疫。

肺移植是几种终末期肺部疾病的挽救生命的疗法。但是，肺同种异体移植的存活率最低，主要是由于排斥。已经广泛研究了同种免疫的发病机理及其在同种异体移植排斥中的作用，并描述了多种诱导耐受的方法。然而，在肺移植的背景下，维持对自身抗原的耐受性的机制失调可导致肺限制性自身免疫，最近已发现其可驱动同种异体移植排斥反应的免疫机制。确实，既存的以及从头进行的肺限制性自身免疫均可在同种异体肺移植排斥反应的发展中发挥重要作用。三种研究最广泛的肺限制性自身抗原包括I型胶原蛋白，V型胶原蛋白，和k-alpha 1微管蛋白 在这篇综述中，我们讨论了肺限制性自身免疫在早期和晚期同种异体移植排斥反应发展中的作用，并且最近的文献通过维持外周耐受性的免疫机制的功能障碍提供了对肺限制性自身免疫发展的见识。