Rationale: Some with interstitial lung abnormalities (ILA) are suspected to have interstitial lung disease (ILD), a subgroup with adverse outcomes. Rates of development and progression of suspected ILD and their effect on mortality are unknown. Objectives: To determine rates of development, progression, and mortality in those with suspected ILD and assess effects of individual ILD and progression criteria. Methods: Participants from COPDGene (Genetic Epidemiology of Chronic Obstructive Pulmonary Disease) with ILA characterization and FVC at enrollment and 5-year follow-up were included. ILD was defined as ILA and fibrosis and/or FVC < 80% predicted. Prevalent ILD was assessed at enrollment and incident ILD and progression were assessed at 5-year follow-up. Computed tomography (CT) progression was assessed visually and FVC decline as relative change. Multivariable Cox regression tested associations between mortality and prevalent ILD, incident ILD, and progression groups. Measurements and Main Results: Of 9,588 participants at enrollment, 268 (2.8%; 51% of ILA) had prevalent ILD. Those with prevalent ILD had 51% mortality after median 10.6 years, which was higher than those with ILA without prevalent ILD (henceforth ILA) (33%; hazard ratio [HR], 2.0; P < 0.001). The subgroup of prevalent ILD with only fibrosis criteria (FVC ≥ 80%) had worse mortality (58%) than ILA (HR, 2.2; P < 0.001). A total of 98 participants with prevalent ILD completed 5-year follow-up: 33% had stable CT and relative FVC decline <10%, 6% had FVC decline ≥10% only, 39% had CT progression only, and 22% had both CT progression and FVC decline ≥10%. Mortality rates were 31%, 50%, 45%, and 45%, respectively; those with only CT progression had worse mortality than those with ILA (HR, 2.6; P = 0.005). At 5-year follow-up, incident ILD occurred in 148/4,842 participants without prevalent ILD (5.5/1,000 person-years) and had worse mortality than ILA (HR, 2.4; P < 0.001). Conclusion: Rates of mortality and progression are high among those with suspected ILD in COPDGene; fibrosis and radiologic progression are important predictors of mortality.

中文翻译：

---

COPDGene 中疑似间质性肺病的发生、进展和死亡率。


理由：一些患有间质性肺异常 （ILA） 的人被怀疑患有间质性肺病 （ILD），这是一个具有不良后果的亚组。疑似 ILD 的发生和进展速度及其对死亡率的影响尚不清楚。研究目的： 确定疑似 ILD 患者的发生率、进展率和死亡率，并评估个体 ILD 和进展标准的影响。方法： 包括来自 COPDGene （慢性阻塞性肺病遗传流行病学） 的参与者，在入组和 5 年随访时具有 ILA 特征和 FVC。ILD 定义为 ILA 和纤维化和/或 FVC < 预测值为 80%。在入组时评估患病 ILD，并在 5 年随访时评估新发 ILD 和进展。目测评估计算机断层扫描 （CT） 进展，FVC 下降为相对变化。多变量 Cox 回归测试了死亡率与患病 ILD 、新发 ILD 和进展组之间的关联。测量和主要结果： 在入组的 9,588 名参与者中，268 名 （2.8%;51% 的 ILA） 患有普遍的 ILD。患有 ILD 患病的患者在中位 10.6 年后死亡率为 51%，高于没有患病 ILD 的 ILA 患者（以下简称 ILA）（33%;风险比 [HR]，2.0;P < 0.001）。仅具有纤维化标准 （FVC ≥ 80%） 的流行 ILD 亚组的死亡率 （58%） 低于 ILA （HR，2.2;P < 0.001）。共有 98 名患有普遍 ILD 的参与者完成了 5 年随访：33% 的 CT 稳定且 FVC 相对下降 <10%，6% 的 FVC 下降≥仅 10%，39% 仅 CT 进展，22% 的 CT 进展和 FVC 下降 ≥10%。死亡率分别为 31% 、 50% 、 45% 和 45% ;仅 CT 进展的患者死亡率低于 ILA 患者 （HR，2.6;P = 0。在 5 年随访中，148/4,842 名没有普遍 ILD 的参与者（5.5/1,000 人年）发生 ILD，死亡率比 ILA 差（HR，2.4;P < 0.001）。结论： COPDGene 疑似 ILD 患者的死亡率和进展率较高;纤维化和放射学进展是死亡率的重要预测指标。