Department of Medicine and Surgery, University of Milan Bicocca, Respiratory Unit, Fondazione IRCCS San Gerardo Dei Tintori, Monza, Italy.
Division of Respirology, Western University, London, Canada.
Lung. 2024 Dec;202(6):793-800. doi: 10.1007/s00408-024-00748-5. Epub 2024 Sep 20.
Fibrosing interstitial lung diseases (ILDs) often progress despite treatment and become life-threatening, with lung transplant (LTx) remaining the only curative option. Six-minute walk distance (6MWD) is increasingly recognized as reliable predictor of clinical course, especially when longitudinally considered. The use of reference equations to express 6MWD as percent predicted (6MWD%) has not been previously studied in fibrosing ILDs. We sought to investigate whether the prognostic power of 6MWD% is superior to that of 6MWD expressed in meters (6MWD-m).
A retrospective, multicenter cohort analysis was conducted on both idiopathic pulmonary (IPF) and non-IPF fibrosing ILD patients. Patients were divided into a discovery (n = 211) and a validation (n = 260) cohort. Longitudinal changes of 6MWD% and lung function parameters were simultaneously considered. LTx-free survival at 3 years from baseline was the endpoint. Competing risks of death and LTx were considered.
Baseline 6MWD% and its longitudinal changes were significant predictors of LTx-free survival and independent from lung function variables. In both cohorts, on multivariate cox proportional hazard regression analysis, receiver operating characteristics analysis and Kaplan-Meier estimates, 6MWD% was consistently, but only slightly superior to 6MWD-m as a predictor of LTx-free survival.
6MWD% has only a slight, yet detectable advantage over 6MWD-m as a predictor of survival in fibrosing ILDs. Utilizing 6MWD% may aid in risk stratification, treatment monitoring, and LTx timing optimization. However, available reference equations do have predicting limitations. Refined predictive equations and standardizing reporting practices are therefore needed to further enhance the clinical utility of 6MWD% in fibrosing ILDs.
纤维化间质性肺病 (ILD) 常因治疗而进展,危及生命,肺移植 (LTx) 仍是唯一的根治选择。六分钟步行距离 (6MWD) 越来越被认为是临床病程的可靠预测指标,尤其是当进行纵向考虑时。使用参考方程将 6MWD 表示为预测百分比 (6MWD%) 在纤维化 ILD 中尚未得到研究。我们旨在研究 6MWD% 的预后能力是否优于以米表示的 6MWD (6MWD-m)。
对特发性肺纤维化 (IPF) 和非特发性纤维化 ILD 患者进行回顾性、多中心队列分析。患者分为发现队列 (n=211) 和验证队列 (n=260)。同时考虑了 6MWD% 和肺功能参数的纵向变化。从基线开始的 3 年无 LTx 生存率是终点。考虑了死亡和 LTx 的竞争风险。
基线 6MWD%及其纵向变化是 LTx 无生存的显著预测因子,与肺功能变量独立。在两个队列中,在多变量 Cox 比例风险回归分析、受试者工作特征分析和 Kaplan-Meier 估计中,6MWD% 在预测 LTx 无生存方面始终略优于 6MWD-m。
6MWD% 作为纤维化 ILD 生存预测指标,仅具有轻微但可检测的优势。使用 6MWD% 可能有助于风险分层、治疗监测和 LTx 时机优化。然而,现有的参考方程确实具有预测局限性。因此,需要改进预测方程并规范报告实践,以进一步提高 6MWD% 在纤维化 ILD 中的临床实用性。