Validation of Proposed Criteria for Progressive Pulmonary Fibrosis
Validation of Proposed Criteria for Progressive Pulmonary Fibrosis
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DOI：
10.1164/rccm.202201-0124oc
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发表时间：
2023-01-01
影响因子：
24.7
通讯作者：
Oldham, Justin M.
中科院分区：
文献类型：
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作者：
Pugashetti, Janelle Vu;Adegunsoye, Ayodeji;Oldham, Justin M.
理论基础：进行性肺纤维化(PPF)的标准已被提出，但其预后价值除了FVC的绝对下降外尚不清楚。目的：确定所建议的PPF标准是否可预测非特发性肺纤维化(IPF)形式间质性肺疾病(ILD)患者的无移植生存(TFS)。来自三个美国中心和一个英国中心的被诊断为纤维结缔组织疾病相关性ILD、纤维过敏性肺炎和非IPF特发性间质性肺炎的患者分别构成了测试和验证队列。使用COX比例风险回归来检验5年TFS和>；=10%FVC下降之间的关系，然后是在没有>；=10%FVC下降的情况下满足的另外13个PPF标准。测量和主要结果：1,341名患者符合纳入标准。A>；=10%的相对FVC下降是TFS降低的最强预测因子，并在队列、ILD亚型和治疗组之间显示出一致的TFS相关性，导致了与IPF非常相似的表型。在美国测试队列中，在没有10%的相对FVC下降的情况下满足的另外10个PPF标准也与TFS的减少有关，英国验证队列中有6个保持TFS相关性。需要结合生理、放射和症状恶化的有效PPF标准与其独立组成部分的表现相似，但纳入的患者数量较少。结论：FVC下降=10%和在没有这种下降的情况下满足的另外6个PPF标准确定了非IPF ILD患者死亡或肺移植的风险增加。
Rationale: Criteria for progressive pulmonary fibrosis (PPF) have been proposed, but their prognostic value beyond categorical decline in FVC remains unclear.Objectives: To determine whether proposed PPF criteria predict transplant-free survival (TFS) in patients with non-idiopathic pulmonary fibrosis (IPF) forms of interstitial lung disease (ILD).Methods: A retrospective, multicenter cohort analysis was performed. Patients with diagnoses of fibrotic connective tissue disease-associated ILD, fibrotic hypersensitivity pneumonitis, and non-IPF idiopathic interstitial pneumonia from three U.S. centers and one UK center constituted the test and validation cohorts, respectively. Cox proportional hazards regression was used to test the association between 5-year TFS and >= 10% FVC decline, followed by 13 additional PPF criteria satisfied in the absence of >= 10% FVC decline.Measurements and Main Results: One thousand three hundred forty-one patients met the inclusion criteria. A >= 10% relative FVC decline was the strongest predictor of reduced TFS and showed consistent TFS association across cohorts, ILD subtypes, and treatment groups, resulting in a phenotype that closely resembled IPF. Ten additional PPF criteria satisfied in the absence of 10% relative FVC decline were also associated with reduced TFS in the U.S. test cohort, with 6 maintaining TFS associations in the UK validation cohort. Validated PPF criteria requiring a combination of physiologic, radiologic, and symptomatic worsening performed similarly to their stand-alone components but captured a smaller number of patients.Conclusions: An FVC decline of >= 10% and six additional PPF criteria satisfied in the absence of such decline identify patients with non-IPF ILD at increased risk for death or lung transplantation.