Lung transplantation (LTx) is the final treatment option for patients with endstage lung diseases including chronic obstructive pulmonary disease, cystic fibrosis, and interstitial lung disease. Survival after LTx is severely hampered by the development of the bronchiolitis obliterans syndrome (BOS) which is hallmarked by excessive fibrosis and scar tissue formation leading to small airway obliteration and eventually organ failure. The pathophysiology of BOS is incompletely understood. During the past years both anti-HLA and non-HLA antibodies have been identified that correlate with transplantation outcome. Also, the involvement of autoimmunity on BOS progression has been demonstrated, including autoantigens Type V collagen and K-alpha tubulin. Both allo- and autoantibodies binding to its respective antigen trigger the binding of C1q and sequential complement activation which can lead to either cell damage or activation, both processes which fit into the current model of BOS pathogenesis. In this review we will discuss both HLA, non-HLA and autoantibodies associated with disease progression, but also elaborate on the subsequent complement effector mechanisms, complement regulation, and the potential influence of regulatory mechanisms on graft survival.

中文翻译：

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肺移植后的体液免疫和补体效应器机制。

肺移植（LTx）是终末期肺部疾病（包括慢性阻塞性肺病，囊性纤维化和间质性肺病）患者的最终治疗选择。闭塞性细支气管炎综合征（BOS）的发展严重阻碍了LTx的生存，其特征是过度纤维化和形成疤痕组织导致细小气道闭塞，最终导致器官衰竭。BOS的病理生理学尚未完全了解。在过去的几年中，已经确定了与移植结果相关的抗HLA和非HLA抗体。同样，已经证明了自身免疫与BOS进展有关，包括自身抗原V型胶原蛋白和K-α微管蛋白。与自身抗原结合的同种抗体和自身抗体都触发C1q的结合和顺序补体激活，这可能导致细胞损伤或激活，这两个过程都适合当前的BOS发病机理模型。在这篇综述中，我们将讨论与疾病进展相关的HLA，非HLA和自身抗体，还详细介绍了随后的补体效应机制，补体调节以及调节机制对移植物存活的潜在影响。