Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico.
Primary Systemic Vasculitides Clinic, Instituto Nacional de Enfermedades Respiratorias, Mexico City, Mexico.
Autoimmun Rev. 2021 Apr;20(4):102781. doi: 10.1016/j.autrev.2021.102781. Epub 2021 Feb 18.
ANCA-associated vasculitides (AAV) comprise three diseases: granulomatosis with polyangiitis, microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. They are characterised by small vessel inflammation and have a broad range of clinical manifestations and multiorgan involvement which endanger the patient's life. An increasingly recognised complication of AAV, especially in MPA is lung fibrosis, for which no clearcut therapy in this context is available. The release of neutrophil extracellular traps (NETs) in these diseases has been related to the development of fibrosis, but the precise mechanisms are not fully unravelled. This review provides an overview of some of the important proteins known to compose NETs, and proposes some mechanisms by which these remarkable components may exert an impact on the different fibroblastic phenotypes leading to lung fibrosis.
抗中性粒细胞胞浆抗体相关性血管炎（AAV）包括三种疾病：肉芽肿性多血管炎、显微镜下多血管炎（MPA）和嗜酸性肉芽肿性多血管炎。它们的特征是小血管炎症，具有广泛的临床表现和多器官受累，危及患者的生命。AAV 的一种越来越被认识到的并发症，特别是在 MPA 中，是肺纤维化，在这种情况下，没有明确的治疗方法。这些疾病中中性粒细胞胞外陷阱（NETs）的释放与纤维化的发展有关，但确切的机制尚未完全阐明。这篇综述概述了一些已知构成 NETs 的重要蛋白质，并提出了这些显著成分可能通过哪些机制对导致肺纤维化的不同成纤维细胞表型产生影响。
