Hôpital Louis Pradel, Centre Coordinateur National de Référence des Maladies Pulmonaires Rares, Hospices Civils de Lyon, UMR754, Université de Lyon, INRAE, OrphaLung, RespiFil, Lyon, France.
Inserm U933, RaDiCo, French National Program on Rare Disease Cohorts, Hôpital Trousseau, Paris, France.
Respiration. 2022;101(1):34-45. doi: 10.1159/000518008. Epub 2021 Sep 2.
There is growing evidence of gender-specific phenotypic differences among patients with idiopathic pulmonary fibrosis (IPF), which may affect patient outcomes.
We present the characteristics of patients with IPF at inclusion in the French Rare Disease Cohort - Interstitial Lung Disease (RaDiCo-ILD) with the aim of characterizing gender-specific phenotypic differences.
Patients with IPF who were enrolled in the national, multicentre RaDiCo-ILD cohort were included. Demographic characteristics, comorbidities, health-related quality of life (HRQoL) scores, pulmonary function, chest imaging, and IPF treatment were collected at inclusion and described by gender.
The cohort included 724 patients with IPF (54% of RaDiCo-ILD cohort), of whom 82.9% were male. The proportion of male and female patients with a prior history of smoking was 75.0% and 26.8%, respectively. Emphysema was present in 17.0% (95% confidence interval [CI]: 10.0, 24.0) of men and 5.4% (95% CI: 1.2, 9.6) of women. At inclusion, females had poorer HRQoL than males based on St. George's Respiratory Questionnaire scores (48.5 [95% CI: 43.9, 53.0] and 41.5 [39.4, 43.6], respectively). The mean forced vital capacity per cent predicted was 77.7% (95% CI: 76.2, 79.3) and 87.4% (83.4, 91.4) for males and females, respectively. Honeycombing on high-resolution computed tomography (HRCT) was present in 70.8% (95% CI: 61.0, 80.6) of males and 45.8% (95% CI: 35.1, 56.5) of females.
This analysis of patients with IPF at inclusion in the RaDiCo-ILD cohort provides evidence that comorbid emphysema, lung volume reduction, and honeycombing on HRCT are more common characteristics of males than females.
特发性肺纤维化(IPF)患者存在越来越多的性别特异性表型差异,这可能会影响患者的结局。
我们介绍了法国罕见病队列-间质性肺病(RaDiCo-ILD)中纳入的 IPF 患者的特征,旨在描述性别特异性表型差异。
纳入了参加全国多中心 RaDiCo-ILD 队列的 IPF 患者。收集患者的人口统计学特征、合并症、健康相关生活质量(HRQoL)评分、肺功能、胸部影像学和 IPF 治疗情况,并按性别进行描述。
该队列纳入了 724 例 IPF 患者(RaDiCo-ILD 队列的 54%),其中 82.9%为男性。有吸烟史的男性和女性患者的比例分别为 75.0%和 26.8%。肺气肿在男性中的占比为 17.0%(95%可信区间[CI]:10.0,24.0),女性为 5.4%(95% CI:1.2,9.6)。纳入时,女性的圣乔治呼吸问卷评分(48.5 [95% CI:43.9,53.0]和 41.5 [39.4,43.6])比男性差。男性和女性的预计用力肺活量百分比分别为 77.7%(95% CI:76.2,79.3)和 87.4%(83.4,91.4)。高分辨率计算机断层扫描(HRCT)上的蜂巢征在男性中的占比为 70.8%(95% CI:61.0,80.6),女性为 45.8%(95% CI:35.1,56.5)。
该分析显示,在 RaDiCo-ILD 队列中纳入的 IPF 患者中,合并肺气肿、肺容积减少和 HRCT 上的蜂巢征在男性中比女性更常见。