Idiopathic pulmonary fibrosis (IPF), the most lethal form of interstitial pneumonia of unknown cause, is associated with a specific radiological or histopathological pattern (the so-called usual interstitial pneumonia pattern) and has a median survival estimated to be between 3 and 5 years after diagnosis.¹ However, evidence shows that IPF has different clinical phenotypes, which are characterised by a variable disease course over time.² At present, the natural history of IPF is unpredictable for individual patients, although some genetic factors and circulating biomarkers have been associated with different prognoses.

中文翻译：

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预测特发性肺纤维化患者的未来：向前迈出的又一步

特发性肺纤维化（IPF）是原因不明的间质性肺炎最致命的形式，与特定的放射学或组织病理学模式（所谓的通常的间质性肺炎模式）相关，中位生存期估计为3至5年诊断后。¹但是，有证据表明IPF具有不同的临床表型，其特征是随着时间的推移会改变病程。²目前，尽管某些遗传因素和循环生物标志物已与不同的预后相关，但个别患者的IPF的自然病程尚无法预测。