Division of Pulmonary and Critical Care Medicine, Department of Medicine.
Division of General Pediatrics, Department of Pediatrics, and.
Ann Am Thorac Soc. 2023 Jan;20(1):30-37. doi: 10.1513/AnnalsATS.202204-328OC.
Historically, sarcoidosis was described as a restrictive lung disease, but several alternative phenotypes of pulmonary function have been observed. Pulmonary function phenotypes in sarcoidosis may represent different clinical and/or molecular phenotypes. To characterize the prevalence of different pulmonary function phenotypes in a large and diverse sarcoidosis cohort from a tertiary care referral center. We identified individuals seen between 2005-2015 with a confirmed diagnosis of sarcoidosis. Data were collected from the first pulmonary function test (PFT) performed at our institution which included spirometry and diffusing capacity of the lung for carbon monoxide (Dl). Demographics and clinical data were collected. Chi-squared analyses and multiple linear regressions were done to assess statistical differences and associations. Global Lung Function Initiative equations were used to calculate percent predicted measurements for spirometry and Dl. Of 602 individuals with sarcoidosis, 93% (562) had pulmonary involvement, 64% (385) were female, and 57% (341) were Black. Of those with pulmonary involvement, 56% had abnormal pulmonary function. Lung function impairment phenotypes included: 47% restriction, 22% obstruction, 15% isolated reduction in Dl, and 16% combined obstructive restrictive phenotype. Restriction was the most common PFT phenotype among Black individuals (41%), while no lung impairment was most common among White individuals (66%) ( < 0.001). Males more frequently had obstruction (19%) compared with females (9%) = 0.001, and females had more restriction (30%) compared with males (21%) = 0.031. Among individuals with sarcoidosis and pulmonary function impairment, less than half demonstrated a restrictive phenotype. There were significant differences in pulmonary function phenotypes by race and sex.
从历史上看,结节病被描述为一种限制性肺病,但也观察到了几种替代的肺功能表型。结节病的肺功能表型可能代表不同的临床和/或分子表型。为了描述来自三级转诊中心的大型和多样化结节病队列中不同肺功能表型的患病率。我们确定了 2005-2015 年间在我们机构就诊并确诊为结节病的个体。从我们机构进行的第一次肺功能测试(PFT)中收集数据,其中包括肺活量测定和一氧化碳弥散量(Dl)。收集人口统计学和临床数据。进行卡方分析和多元线性回归,以评估统计差异和关联。使用全球肺功能倡议方程计算肺活量测定和 Dl 的预计百分比测量值。在 602 名患有结节病的患者中,93%(562 名)有肺部受累,64%(385 名)为女性,57%(341 名)为黑人。在有肺部受累的患者中,56%的肺功能异常。肺功能损伤表型包括:47%限制,22%阻塞,15%孤立性 Dl 降低,16%混合性阻塞性限制表型。限制是黑人中最常见的 PFT 表型(41%),而白人中最常见的是无肺损伤(66%)( < 0.001)。男性比女性更常发生阻塞(19%比 9%) = 0.001,女性比男性更常发生限制(30%比 21%) = 0.031。在有肺功能障碍的结节病患者中,不到一半表现为限制性表型。肺功能表型在种族和性别上存在显著差异。