Karampitsakos Theodoros, Tourki Bochra, Herazo-Maya Jose D
Division of Pulmonary, Critical Care and Sleep Medicine, Ubben Center for Pulmonary Fibrosis Research, Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL.
Division of Pulmonary, Critical Care and Sleep Medicine, Ubben Center for Pulmonary Fibrosis Research, Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL.
Chest. 2025 Apr;167(4):1120-1132. doi: 10.1016/j.chest.2024.10.042. Epub 2024 Nov 8.
Interstitial lung diseases (ILDs) represent a broad group of heterogeneous parenchymal lung diseases. Some ILDs progress, causing architectural distortion and pulmonary fibrosis, and thus are called fibrotic ILDs. Recent studies have shown a beneficial effect of antifibrotic therapy in fibrotic ILDs other than idiopathic pulmonary fibrosis (IPF) that manifest progressive pulmonary fibrosis (PPF). However, it remains challenging to predict which patients with fibrotic ILDs will demonstrate PPF. Precision medicine approaches could identify patients at risk for progression and guide treatment in patients with IPF or PPF.
Multiple biomarkers able to highlight disease susceptibility risk, to provide an accurate diagnosis, and to prognosticate or assess treatment response have been identified. Advances in precision medicine led to the identification of endotypes that could discriminate patients with different fibrotic ILDs or patients with different disease courses. Importantly, recent studies have shown that particular compounds were efficacious only in particular endotypes. The aforementioned findings are promising. However, implementation in clinical practice remains an unmet need.
Substantial progress has been observed in the context of precision medicine approaches in fibrotic ILDs in recent years. Nonetheless, infrastructure, financial, regulatory, and ethical challenges remain before precision medicine can be implemented in clinical practice. Overcoming such barriers and moving from a one-size-fits-all approach to patient-centered care could improve patient quality of life and survival substantially.
间质性肺疾病（ILDs）是一大类异质性的实质性肺疾病。一些ILDs会进展，导致肺结构破坏和肺纤维化，因此被称为纤维化ILDs。最近的研究表明，抗纤维化治疗对除特发性肺纤维化（IPF）以外表现为进行性肺纤维化（PPF）的纤维化ILDs有有益作用。然而，预测哪些纤维化ILDs患者会出现PPF仍然具有挑战性。精准医学方法可以识别有疾病进展风险的患者，并指导IPF或PPF患者的治疗。
已经确定了多种能够突出疾病易感性风险、提供准确诊断以及预测或评估治疗反应的生物标志物。精准医学的进展导致了内型的识别，这些内型可以区分不同纤维化ILDs患者或不同病程患者。重要的是，最近的研究表明，特定化合物仅在特定内型中有效。上述发现很有前景。然而，在临床实践中的应用仍然是未满足的需求。
近年来，在纤维化ILDs的精准医学方法方面已经取得了实质性进展。尽管如此，在精准医学能够在临床实践中实施之前，基础设施、资金、监管和伦理方面的挑战仍然存在。克服这些障碍，从一刀切的方法转向以患者为中心的护理，可以显著提高患者的生活质量和生存率。
