De Sadeleer Laurens J, Goos Tinne, Yserbyt Jonas, Wuyts Wim A
Laboratory of Respiratory Diseases and Thoracic Surgery (BREATHE), Department CHROMETA, KU Leuven, B-3000 Leuven, Belgium.
Unit of Interstitial Lung Diseases, Department of Respiratory Diseases, University Hospitals Leuven, B-3000 Leuven, Belgium.
J Clin Med. 2020 Jun 3;9(6):1722. doi: 10.3390/jcm9061722.
Although only recently introduced in the ILD community, the concept of progressive fibrosing interstitial lung disease (PF-ILD) has rapidly acquired an important place in the management of non-idiopathic pulmonary fibrosis fibrosing ILD (nonIPF fILD) patients. It confirms a clinical gut feeling that an important subgroup of nonIPF fILD portends a dismal prognosis despite therapeutically addressing the alleged triggering event. Due to several recently published landmark papers showing a treatment benefit with currently available antifibrotic drugs in PF-ILD patients, endorsing a PF-ILD phenotype has vital therapeutic consequences. Importantly, defining progressiveness is based on former progression, which has proven to be a rather moderate predictor of future progression. As fibrosis extent >20% and the presence of honeycombing have superior predictive properties regarding future progression, we advocate immediate initiation of antifibrotic treatment in the presence of these risk factors. In this perspective, we describe the historical context wherein PF-ILD has emerged, determine the currently employed PF-ILD criteria and their inherent limitations and propose new directions to mature its definition. Finally, while ascertaining progression in a nonIPF fILD patient clearly demonstrates the need for (additional) therapy, in the future, therapeutic decisions should be taken after assessing which pathway is ultimately driving the progression. Although not readily available, pathophysiological insight and diagnostic means are emergent to go full steam ahead in this novel direction.
尽管进行性纤维化间质性肺疾病（PF-ILD）的概念最近才在间质性肺疾病（ILD）领域被引入，但它已迅速在非特发性肺纤维化的纤维化ILD（nonIPF fILD）患者管理中占据重要地位。它证实了一种临床直觉，即尽管对所谓的触发事件进行了治疗，但nonIPF fILD的一个重要亚组预后不佳。由于最近发表的几篇具有里程碑意义的论文显示，目前可用的抗纤维化药物对PF-ILD患者有治疗益处，认可PF-ILD表型具有至关重要的治疗意义。重要的是，定义疾病进展是基于既往的进展情况，而这已被证明是未来进展的一个相当一般的预测指标。由于纤维化范围>20%和蜂窝状改变对未来进展具有更好的预测价值，我们主张在存在这些危险因素时立即启动抗纤维化治疗。从这个角度出发，我们描述了PF-ILD出现的历史背景，确定了目前采用的PF-ILD标准及其固有的局限性，并提出了完善其定义的新方向。最后，虽然确定nonIPF fILD患者的疾病进展清楚地表明需要（额外的）治疗，但未来在评估最终驱动疾病进展的途径后应做出治疗决策。尽管目前尚不容易获得，但病理生理学见解和诊断手段对于在这个新方向上全力推进至关重要。
