BACKGROUND Limited data are available on the association between clinically measured disease severity markers and quality of life (QOL) in idiopathic pulmonary fibrosis (IPF). We examined the associations between objective disease severity metrics and QOL in a contemporary IPF population. METHODS We evaluated baseline data from patients enrolled in the multicenter, US-based IPF-PRO Registry between June 2014 and July 2018. Disease severity metrics included forced vital capacity [FVC] % predicted, diffusing capacity for carbon monoxide [DLCO] % predicted, supplemental oxygen use with activity, supplemental oxygen use at rest, and two summary scores: the Gender-Age-Lung Physiology (GAP) Index (based on gender, age, and % predicted values for DLCO and FVC) and the Composite Physiologic Index (CPI) (based on % predicted values for DLCO, FVC, and forced expiratory volume in 1 second). We used multivariable adjusted regression models to examine cross-sectional associations between each severity measure and St. George's Respiratory Questionnaire (SGRQ) total score. RESULTS Among 829 patients with complete SGRQ data, the median SGRQ score at enrollment was 40 (IQR=26-53; higher scores indicate worse QOL). Modest SGRQ impairments were observed with increasing GAP score [2.9 (1.8-4.0) per 1-point increase] and with increasing CPI [3.0 (2.4-3.6) per 5-point increase]. Substantial SGRQ impairments were observed for oxygen use with activity [15.6 (12.9-18.2)], oxygen use at rest [16.2 (13.0-19.4)], and decreasing DLCO [5.0 (4.0-6.1) per 10% decrease in % predicted]. CONCLUSIONS Objective measures of disease severity, including severity scores, physiologic parameters, and supplemental oxygen use, are associated with worse QOL in patients with IPF.

中文翻译：

---

特发性肺纤维化患者的疾病严重程度和生活质量：IPF-PRO 注册的横断面分析

背景 关于临床测量的疾病严重程度标志物与特发性肺纤维化 (IPF) 的生活质量 (QOL) 之间关联的可用数据有限。我们检查了当代 IPF 人群中客观疾病严重程度指标与 QOL 之间的关联。方法 我们评估了 2014 年 6 月至 2018 年 7 月期间在美国多中心 IPF-PRO 登记处登记的患者的基线数据。疾病严重程度指标包括用力肺活量 [FVC] 预测百分比、一氧化碳扩散能力 [DLCO] 预测百分比，活动时补充氧气使用、休息时补充氧气使用和两个总分：性别-年龄-肺生理 (GAP) 指数（基于性别、年龄和 DLCO 和 FVC 的预测值百分比）和综合生理指数（ CPI)（基于 DLCO、FVC、1 秒内用力呼气量）。我们使用多变量调整回归模型来检查每个严重程度测量与圣乔治呼吸问卷 (SGRQ) 总分之间的横断面关联。结果 在 829 名具有完整 SGRQ 数据的患者中，入组时的中位 SGRQ 得分为 40（IQR=26-53；得分越高表示 QOL 越差）。随着 GAP 分数的增加 [每增加 1 点为 2.9 (1.8-4.0)] 和增加 CPI [每增加 5 点为 3.0 (2.4-3.6)]，观察到适度的 SGRQ 损伤。观察到活动的氧气使用 [15.6 (12.9-18.2)]、休息时的氧气使用 [16.2 (13.0-19.4)] 和 DLCO [5.0 (4.0-6.1) 每减少 10% 预测百分比] 的显着 SGRQ 损伤] . 结论 疾病严重程度的客观测量，包括严重程度评分、生理参数、