系统性硬化症中小气道疾病的相关临床特征。

Clinical characteristics associated with small airways disease in systemic sclerosis.

作者信息

Varma Sanskriti, Yun Jae Hee, Kim John S, Podolanczuk Anna J, Patel Nina M, Bernstein Elana J

机构信息

Department of Medicine, NewYork-Presbyterian and Columbia University Irving Medical Center, New York, NY, USA.

Department of Medicine, School of Medicine, University of Virginia, Charlottesville, VA, USA.

出版信息

J Scleroderma Relat Disord. 2022 Jun;7(2):128-134. doi: 10.1177/23971983221083882. Epub 2022 Mar 20.

DOI:10.1177/23971983221083882

PMID:35585957

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9109501/

Abstract

OBJECTIVE

Pulmonary manifestations of systemic sclerosis are a major cause of morbidity and mortality. Small airways disease can cause dyspnea and pulmonary function test abnormalities. We aimed to determine the prevalence of small airways disease and describe the characteristics associated with small airways disease in a cohort of systemic sclerosis patients.

METHODS

We performed a retrospective cohort study of adults with systemic sclerosis who met American College of Rheumatology/European League Against Rheumatism 2013 classification criteria and were evaluated at our institution between November 2000 and November 2015. Patients with prior lung transplantation were excluded. Small airways disease was defined as the presence of one or more of the following: airway-centered fibrosis on surgical lung biopsy, forced expiratory volume at 25-75% ⩽ 50% on pulmonary function tests, and/or high-resolution computed tomography scan of the chest with bronchiolitis, mosaic attenuation, or air trapping on expiratory views. The primary outcome was small airways disease diagnosis. We performed multivariable logistic regression to determine the association of clinical variables with small airways disease.

RESULTS

One-hundred thirty-six systemic sclerosis patients were included; 55 (40%) had small airways disease. Compared to those without small airways disease, a significantly greater proportion of those with small airways disease had interstitial lung disease, chronic obstructive pulmonary disease, pulmonary hypertension, and gastroesophageal reflux disease. On multivariable analysis, pulmonary hypertension (odds ratio = 2.91, 95% confidence interval = 1.11-7.65, p-value = 0.03), gastroesophageal reflux disease (odds ratio = 2.70, 95% confidence interval = 1.08-6.79, p-value = 0.034), and anti-topoisomerase I (anti-Scl-70) antibody positivity (odds ratio = 0.42, 95% confidence interval = 0.19-0.93, p-value = 0.033) were associated with diagnosis of small airways disease.

CONCLUSION

Small airways disease is prevalent among systemic sclerosis patients; those with pulmonary hypertension or gastroesophageal reflux disease may have a higher risk of small airways disease.

摘要

目的

系统性硬化症的肺部表现是发病和死亡的主要原因。小气道疾病可导致呼吸困难和肺功能测试异常。我们旨在确定系统性硬化症患者队列中小气道疾病的患病率，并描述与小气道疾病相关的特征。

方法

我们对符合美国风湿病学会/欧洲抗风湿病联盟2013年分类标准且于2000年11月至2015年11月在我们机构接受评估的成年系统性硬化症患者进行了一项回顾性队列研究。排除先前接受过肺移植的患者。小气道疾病定义为存在以下一种或多种情况：手术肺活检显示以气道为中心的纤维化、肺功能测试中25%至75%的用力呼气量≤50%，和/或胸部高分辨率计算机断层扫描显示细支气管炎、马赛克样衰减或呼气期空气潴留。主要结局是小气道疾病诊断。我们进行多变量逻辑回归以确定临床变量与小气道疾病的关联。

结果

纳入了136例系统性硬化症患者；55例（40%）患有小气道疾病。与无小气道疾病的患者相比，患有小气道疾病的患者中，间质性肺疾病、慢性阻塞性肺疾病、肺动脉高压和胃食管反流病的比例显著更高。多变量分析显示，肺动脉高压（比值比=2.91，95%置信区间=1.11-7.65，p值=0.03）、胃食管反流病（比值比=2.70，95%置信区间=1.08-6.79，p值=0.034）和抗拓扑异构酶I（抗Scl-70）抗体阳性（比值比=0.42，95%置信区间=0.19-0.93，p值=0.033）与小气道疾病诊断相关。