Department of Pulmonary and Critical Care Medicine, The Third Affiliated Hospital of Sun Yat-sen University, Institute of Respiratory Diseases of Sun Yat-Sen University, Guangzhou, China.
Clin Respir J. 2023 May;17(5):405-413. doi: 10.1111/crj.13605. Epub 2023 Mar 16.
The pathogenesis of non-cystic fibrosis bronchiectasis has not been clearly clarified. This study aimed to investigate the expression of ciliary regulating protein forkhead box protein j1 (Foxj1) on airway epithelium in non-cystic fibrosis bronchiectasis and its association with airway cilia structure disorder and disease severity.
Lung tissue sections excised from 47 patients with non-cystic fibrosis bronchiectasis were included between January 2018 and June 2021. Specimens from 26 subjects who underwent a lobectomy due to lung nodule were chosen as controls. Clinical information was collected, and pathologic analysis was performed to assess the epithelial structure and expression of ciliary regulating Foxj1.
Of the 47 patients with non-cystic fibrosis bronchiectasis, 25 were considered as mild, 12 were moderate whereas the remaining 10 cases were severe according to the bronchiectasis severity index score evaluation. Epithelial hyperplasia, hyperplasia of goblet cells and inflammatory cell infiltration were observed in non-cystic fibrosis bronchiectasis, compared with control subjects. Cilia length in non-cystic fibrosis bronchiectasis patients were shorter than that in the control group, (5.34 ± 0.89) μm versus (7.34 ± 0.71) μm, respectively (P = 0.002). The expression of Foxj1 was (2.69 ± 1.09) × 10 in non-cystic fibrosis bronchiectasis, compared with (6.67 ± 1.15) × 10 in the control group (P = 0.001). Moreover, patients with lower expression of Foxj1 showed shorter airway cilia and worse in disease severity.
Foxj1 declined in the airway epithelium of patients with non-cystic fibrosis bronchiectasis, positively correlated to cilia length and might imply worse disease severity.
非囊性纤维化性支气管扩张症的发病机制尚未明确。本研究旨在探讨纤毛调节蛋白叉头框蛋白 J1(Foxj1)在非囊性纤维化性支气管扩张症气道上皮中的表达及其与气道纤毛结构紊乱和疾病严重程度的关系。
纳入 2018 年 1 月至 2021 年 6 月期间 47 例非囊性纤维化性支气管扩张症患者的肺组织切片。选择 26 例因肺结节行肺叶切除术的患者作为对照。收集临床资料,进行病理分析,评估上皮结构和纤毛调节 Foxj1 的表达。
根据支气管扩张严重指数评分评估,47 例非囊性纤维化性支气管扩张症患者中,25 例为轻度,12 例为中度,10 例为重度。与对照组相比,非囊性纤维化性支气管扩张症患者存在上皮增生、杯状细胞增生和炎症细胞浸润。非囊性纤维化性支气管扩张症患者的纤毛长度较对照组短,分别为(5.34±0.89)μm和(7.34±0.71)μm(P=0.002)。Foxj1 的表达在非囊性纤维化性支气管扩张症患者中为(2.69±1.09)×10,而在对照组中为(6.67±1.15)×10(P=0.001)。此外,Foxj1 表达水平较低的患者气道纤毛较短,疾病严重程度较差。
非囊性纤维化性支气管扩张症患者气道上皮中的 Foxj1 表达下调,与纤毛长度呈正相关,可能提示疾病严重程度更差。