Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Department of Allergy and Rheumatology, International University of Health and Welfare, Narita, Japan.
Rheumatology (Oxford). 2024 Apr 2;63(4):1162-1171. doi: 10.1093/rheumatology/kead363.
Fibrotic interstitial lung disease (ILD) is a progressive lung disease characterized by loss of lung volume, resulting in a leading cause of death in patients with RA. Crucially, acute exacerbation (AE) of ILD shows higher morbidity and mortality with rapid deterioration of the lungs. However, a quantitative assessment for physiological changes at AE has yet to be performed. This study hypothesized that quantitative assessments of lung volume (LV) accurately indicate disease severity and mortality risk in patients with AE-RA-ILD.
This multicentre cohorts study quantitatively assessed physiological changes of RA-ILD at diagnosis (n = 54), at AE (discovery-cohorts; n = 20, and validation-cohort; n = 33), and controls (n = 35) using 3D CT (3D-CT) images. LV was quantitatively measured using 3D-CT and standardized by predicted forced vital capacity.
Patients with RA-ILD at diagnosis showed decreased LV, predominantly in lower lobes, compared with controls. Further substantial volume loss was found in upper- and lower lobes at AE compared with those at diagnosis. During AE, decreased standardized 3D-CT LV was associated with a worse prognosis in both cohorts. Subsequently, standardized 3D-CT LV was identified as a significant prognostic factor independent of age, sex and the presence of UIP pattern on CT by multivariate analyses. Notably, a composite model of age and standardized 3D-CT LV successfully classified mortality risk in patients with AE-RA-ILD.
Volume loss at AE in patients with RA-ILD was associated with increased mortality. Assessing physiological change using standardized 3D-CT might help evaluate disease severity and mortality risk in patients with AE-RA-ILD.
纤维化间质性肺病（ILD）是一种进行性肺病，其特征是肺容积丧失，导致 RA 患者的主要死亡原因。至关重要的是，ILD 的急性加重（AE）表现出更高的发病率和死亡率，肺部迅速恶化。然而，AE 时的生理变化尚未进行定量评估。本研究假设，对肺容积（LV）的定量评估可以准确指示 AE-RA-ILD 患者的疾病严重程度和死亡风险。
本多中心队列研究使用 3D CT（3D-CT）图像对 RA-ILD 的生理变化进行了定量评估，包括诊断时（n=54）、AE 时（发现队列；n=20，验证队列；n=33）和对照组（n=35）。使用 3D-CT 对 LV 进行定量测量，并通过预测用力肺活量进行标准化。
与对照组相比，RA-ILD 患者在诊断时就表现出 LV 降低，主要在下肺。与诊断时相比，AE 时上、下肺的容积损失更大。在 AE 期间，与诊断时相比，标准化 3D-CT LV 的降低与两个队列的预后更差相关。随后，多变量分析表明，标准化 3D-CT LV 是独立于年龄、性别和 CT 上 UIP 模式的重要预后因素。值得注意的是，年龄和标准化 3D-CT LV 的复合模型成功地对 AE-RA-ILD 患者的死亡风险进行了分类。
RA-ILD 患者在 AE 时的容积损失与死亡率增加相关。使用标准化 3D-CT 评估生理变化可能有助于评估 AE-RA-ILD 患者的疾病严重程度和死亡风险。
