ty -jour t1-当事情出现问题时：探索可能的机制，驱动肺部肺部疾病中的进行性纤维化表型JF-欧洲呼吸杂志塞尔曼（Selman）以肺实质的扩散损伤为特征的已知和未知病因疾病。近年来，具有不同类型ILD的患者有发展进行性肺纤维化的风险，称为进行性纤维化ILD（PF-ILD）。这是一种与特发性肺纤维化相似的表型，这是进行性纤维化的原型例子。PF-ILD不是一个不同的临床实体，而是描述了一组具有相似临床行为的ILD。这种表型可能发生在疾病中，在其启动和发育过程中表现出不同的病因和不同的生物病理学。重要的是，这些实体在进入进行性纤维化阶段之前可能具有改进或稳定的潜力。关键问题是：1）为什么一部分患者即使通过适当的治疗也会发展出进行性和不可逆的纤维化表型？2）驱动进展的可能的致病机制是什么？ Here, we provide a framework highlighting putative mechanisms underlying progression, including genetic susceptibility, ageing, epigenetics, structural fibrotic distortion, aberrant composition and stiffness of the extracellular matrix, and the emergence of distinct pro-fibrotic cell subsets. Understanding the cellular and molecular mechanisms behind PF-ILD will provide the basis for identifying risk factors and appropriate therapeutic strategies.ILDs of different aetiologies, which have the potential for improvement or stabilisation, may develop progressive pulmonary fibrosis (PF-ILD). Mechanisms underlying progression, including (epi)genetics, ageing and structural pattern, are proposed in this review. https://bit.ly/2NSJPXQ ER -