Abstract：
Interstitial lung disease is a common complication of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). It is seen most commonly in microscopic polyangiitis owing to the pathogenic effect of myeloperoxidase in the lung. Oxidative stress, neutrophil elastase release, and expression of inflammatory proteins by neutrophil extracellular traps result in fibroblast proliferation and differentiation and therefore fibrosis. Usually, interstitial pneumonia pattern fibrosis is common and associated with poor survival. Treatment for patients with AAV and interstitial lung disease lacks evidence, and those with vasculitis are treated with immunosuppression, whereas those with progressive fibrosis may well benefit from antifibrotic therapy.
摘要：
间质性肺病是抗中性粒细胞胞浆抗体相关血管炎（AAV）的常见并发症。由于髓过氧化物酶在肺中的致病作用，在显微镜下多血管炎中最常见。氧化应激,中性粒细胞弹性蛋白酶释放，中性粒细胞胞外诱捕器表达的炎性蛋白导致成纤维细胞增殖和分化，从而导致纤维化。通常,间质性肺炎的纤维化模式是常见的，并与生存不良相关。AAV和间质性肺病患者的治疗缺乏证据，血管炎患者接受免疫抑制治疗，而那些进行性纤维化患者可能会从抗纤维化治疗中获益。
