关键词: Rare lung diseases Sarcoidosis

来  源:   DOI:10.1136/thorax-2023-221309

Abstract:
BACKGROUND: Given the heterogeneity of sarcoidosis, predicting disease course of patients remains a challenge. Our aim was to determine whether the 3-year change in pulmonary function differed between pulmonary function phenotypes and whether there were differential longitudinal changes by race and sex.
METHODS: We identified individuals seen between 2005 and 2015 with a confirmed diagnosis of sarcoidosis who had at least two pulmonary function test measurements within 3 years of entry into the cohort. For each individual, spirometry, diffusion capacity, Charlson Comorbidity Index, sarcoidosis organ involvement, diagnosis duration, tobacco use, race, sex, age and medications were recorded. We compared changes in pulmonary function by type of pulmonary function phenotype and for demographic groups.
RESULTS: Of 291 individuals, 59% (173) were female and 54% (156) were black. Individuals with restrictive pulmonary function phenotype had significantly greater 3-year rate of decline of FVC% (forced vital capacity) predicted and FEV1% (forced expiratory volume in 1 s) predicted course when compared with normal phenotype. We identified a subset of individuals in the cohort, highest decliners, who had a median 3-year FVC decline of 156 mL. Black individuals had worse pulmonary function at entry into the cohort measured by FVC% predicted, FEV1% predicted and diffusing capacity for carbon monoxide % predicted compared with white individuals. Black individuals\' pulmonary function remained stable or declined over time, whereas white individuals\' pulmonary function improved over time. There were no sex differences in rate of change in any pulmonary function parameters.
CONCLUSIONS: We found significant differences in 3-year change in pulmonary function among pulmonary function phenotypes and races, but no difference between sexes.
摘要:
背景:鉴于结节病的异质性,预测患者的病程仍然是一个挑战。我们的目的是确定肺功能表型之间的3年肺功能变化是否不同,以及种族和性别之间是否存在不同的纵向变化。
方法:我们确定了2005年至2015年间发现的确诊为结节病的个体,这些个体在进入队列的3年内至少进行了两次肺功能检查。对于每个人来说,肺活量测定,扩散能力,Charlson合并症指数,结节病器官受累,诊断持续时间,烟草使用,种族,性别,记录年龄和用药情况.我们比较了肺功能表型类型和人口统计学组的肺功能变化。
结果:在291个人中,59%(173)为女性,54%(156)为黑人。与正常表型相比,具有限制性肺功能表型的个体预测的FVC%(用力肺活量)和FEV1%(1s内用力呼气量)预测的病程的3年下降率显着更大。我们确定了队列中的一部分个体,下降幅度最高,他们的3年FVC平均下降156mL。黑人个体在进入队列时的肺功能较差,由FVC%预测,与白人个体相比,预测的FEV1%和预测的一氧化碳扩散能力%。黑人个体的肺功能随着时间的推移保持稳定或下降,而白种人的肺功能随着时间的推移而改善。任何肺功能参数的变化率均无性别差异。
结论:我们发现肺功能表型和种族之间的3年肺功能变化存在显着差异,但性别之间没有区别。
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