Monitoring small airway dysfunction in connective tissue disease-related interstitial lung disease: a retrospective and prospective study

Linrui Xu; Giacomo Sgalla; Faping Wang; Min Zhu; Liangyuan Li; Ping Li; Qibing Xie; Xiaoyan Lv; Jianqun Yu; Gang Wang; Huajing Wan; Luca Richeldi; Fengming Luo

doi:10.1186/s12890-023-02381-z

Monitoring small airway dysfunction in connective tissue disease-related interstitial lung disease: a retrospective and prospective study

BMC Pulm Med. 2023 Mar 20;23(1):90. doi: 10.1186/s12890-023-02381-z.

Authors

Linrui Xu^#^{1

2

3}, Giacomo Sgalla^#⁴, Faping Wang^#^{1

2

3}, Min Zhu^#^{1

2

3}, Liangyuan Li^{1

2

3}, Ping Li^{1

2

3}, Qibing Xie⁵, Xiaoyan Lv⁶, Jianqun Yu⁷, Gang Wang^{1

2

3}, Huajing Wan^{8

9

10}, Luca Richeldi¹¹, Fengming Luo^{12

13

14}

Affiliations

¹ Department of Pulmonary and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, P.R. China.
² Laboratory of Pulmonary Immunology and Inflammation, Frontiers Science Center for Disease-Related Molecular Network, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China.
³ Clinical Research Center for Respiratory Disease, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China.
⁴ Division of Pulmonary Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.
⁵ Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China.
⁶ Department of Dermatology, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China.
⁷ Department of Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China.
⁸ Department of Pulmonary and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, P.R. China. wanhuajing1974@wchscu.cn.
⁹ Laboratory of Pulmonary Immunology and Inflammation, Frontiers Science Center for Disease-Related Molecular Network, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China. wanhuajing1974@wchscu.cn.
¹⁰ Clinical Research Center for Respiratory Disease, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China. wanhuajing1974@wchscu.cn.
¹¹ Division of Pulmonary Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy. luca.richeldi@policlinicogemelli.it.
¹² Department of Pulmonary and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, P.R. China. fengmingluo@outlook.com.
¹³ Laboratory of Pulmonary Immunology and Inflammation, Frontiers Science Center for Disease-Related Molecular Network, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China. fengmingluo@outlook.com.
¹⁴ Clinical Research Center for Respiratory Disease, West China Hospital, Sichuan University, Chengdu, Sichuan, P.R. China. fengmingluo@outlook.com.

^# Contributed equally.

Abstract

Background: Small airway dysfunction (SAD), a hallmark of early lung function abnormality, is a major component of several chronic respiratory disorders. The role of SAD in patients with connective tissue disease-related interstitial lung disease (CTD-ILD) has not been explored.

Methods: We conducted a two-parts (retrospective and prospective) study to collect pulmonary function tests from CTD-ILD patients. SAD was defined as at least two of the three measures (MMEF, FEF 50%, and FEF 75%) must be 65% of predicted values. Spearman correlation coefficient was used to evaluate association between SAD and other pulmonary function parameters. Mixed effects regression modeling analysis was used to assess response to treatment.

Results: CTD-ILD patients with SAD and without SAD were compared in this study. In the retrospective study, pulmonary function tests (PFTs) from 491 CTD-ILD patients were evaluated, SAD were identified in 233 (47.5%). CTD-ILD patients with SAD were less smokers (17.6% vs. 27.9%, p = 0.007) and more females (74.3% vs. 64.0%, p = 0.015) than those without SAD. CTD-ILD patients with SAD had lower vital capacity (% predicted FVC, 70.4 ± 18.3 vs. 80.0 ± 20.9, p < 0.001) and lower diffusion capacity (% predicted DLCO, 58.8 ± 19.7 vs. 63.8 ± 22.1, p = 0.011) than those without SAD. Among 87 CTD-ILD patients prospectively enrolled, significant improvement in % predicted FVC was observed at 12-months follow-up (6.37 ± 1.53, p < 0.001 in patients with SAD; 5.13 ± 1.53, p = 0.002 in patients without SAD), but not in diffusion capacity and SAD parameters.

Conclusion: In our cohort, about half of CTD-ILD patients have SAD, which is less frequent in smokers and more common in female patients. CTD-ILD patients with SAD have worse pulmonary function compared to those without SAD. Improvement of FVC but no improvement of SAD was observed in CTD-ILD patients after treatment.

Keywords: Connective tissue disease associated interstitial lung disease; Pulmonary function; Small airway dysfunction; Treatment response.

MeSH terms

Connective Tissue Diseases* / complications
Female
Humans
Lung
Lung Diseases, Interstitial* / etiology
Prospective Studies
Retrospective Studies

Abstract

MeSH terms

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