肺纤维化是肺间质疾病的最终结局,主要累及肺间质、肺泡和细支气管,其病理生理过程包括肺泡上皮细胞的再生失败、持续的成纤维细胞/肌成纤维细胞增殖、细胞外基质沉积、肺结构紊乱失调、有效换气面积减少,最终导致低氧性呼吸衰竭[1]。其发病机制尚不明确,但近年大量研究提示纤维形成和 Pulmonary fibrosis is the ultimate outcome of interstitial lung disease, which mainly affects the pulmonary interstitium, alveoli and bronchioles, and its pathophysiological processes include the failure of alveolar epithelial cells to regenerate, persistent fibroblast / myofibroblast proliferation, extracellular matrix Deposition, dysregulation of lung structure, effective ventilation area decreases, eventually leading to hypoxic respiratory failure [1]. Its pathogenesis is not yet clear, but in recent years a large number of studies suggest that fiber formation and