Department of Internal Medicine V, Ludwig-Maximilian University Munich, Marchioninistrasse 15, 81377, Munich, Germany.
Comprehensive Pneumology Center (CPC-M), Ludwig-Maximilian University, and Helmholtz Center Munich, Member of the German Center for Lung Research (DZL), Munich, Germany.
Respir Res. 2020 Oct 19;21(1):270. doi: 10.1186/s12931-020-01524-8.
Fibrotic interstitial lung disease (ILD) is often associated with poor outcomes, but has few predictors of progression. Daily home spirometry has been proposed to provide important information about the clinical course of idiopathic pulmonary disease (IPF). However, experience is limited, and home spirometry is not a routine component of patient care in ILD. Using home spirometry, we aimed to investigate the predictive potential of daily measurements of forced vital capacity (FVC) in fibrotic ILD.
In this prospective observational study, patients with fibrotic ILD and clinical progression were provided with home spirometers for daily measurements over 6 months. Hospital based spirometry was performed after three and 6 months. Disease progression, defined as death, lung transplantation, acute exacerbation or FVC decline > 10% relative was assessed in the cohort.
From May 2017 until August 2018, we included 47 patients (IPF n = 20; non-IPF n = 27). Sufficient daily measurements were performed by 85.1% of the study cohort. Among these 40 patients (IPF n = 17; non-IPF n = 23), who had a mean ± SD age of 60.7 ± 11.3 years and FVC 64.7 ± 21.7% predicted (2.4 ± 0.8 L), 12 patients experienced disease progression (death: n = 2; lung transplantation: n = 3; acute exacerbation: n = 1; FVC decline > 10%: n = 6). Within the first 28 days, a group of patients had high daily variability in FVC, with 60.0% having a variation ≥5%. Patients with disease progression had significantly higher FVC variability than those in the stable group (median variability 8.6% vs. 4.8%; p = 0.002). Cox regression identified FVC variability as independently associated with disease progression when controlling for multiple confounding variables (hazard ratio: 1.203; 95% CI:1.050-1.378; p = 0.0076).
Daily home spirometry is feasible in IPF and non-IPF ILD and facilitates the identification of FVC variability, which was associated with disease progression.
纤维性间质性肺疾病（ILD）常伴有不良预后，但对其进展的预测因素较少。家庭每日肺活量测定已被提议为特发性肺纤维化（IPF）的临床过程提供重要信息。然而，经验有限，家庭肺活量测定并非ILD 患者护理的常规组成部分。我们使用家庭肺活量测定仪，旨在研究纤维性 ILD 中每日用力肺活量（FVC）测量的预测潜力。
在这项前瞻性观察研究中，为临床进展的纤维性 ILD 患者提供家庭肺活量计进行为期 6 个月的每日测量。在第 3 个月和第 6 个月后进行基于医院的肺活量测定。在队列中评估了定义为死亡、肺移植、急性加重或 FVC 下降>10%的疾病进展。
从 2017 年 5 月至 2018 年 8 月，我们纳入了 47 名患者（IPF n=20；非 IPF n=27）。研究队列中有 85.1%的患者进行了足够的每日测量。在这些 40 名患者（IPF n=17；非 IPF n=23）中，他们的平均年龄为 60.7±11.3 岁，用力肺活量为预测值的 64.7±21.7%（2.4±0.8 升），12 名患者发生疾病进展（死亡：n=2；肺移植：n=3；急性加重：n=1；FVC 下降>10%：n=6）。在最初的 28 天内，一组患者的 FVC 每日变化较大，其中 60.0%的患者变化≥5%。与稳定组相比，疾病进展患者的 FVC 变异性明显更高（中位数变异性 8.6% vs. 4.8%；p=0.002）。Cox 回归分析表明，在控制多种混杂变量后，FVC 变异性与疾病进展独立相关（风险比：1.203；95%CI：1.050-1.378；p=0.0076）。
家庭每日肺活量测定在 IPF 和非 IPF ILD 中是可行的，并有助于识别与疾病进展相关的 FVC 变异性。
