Trajectories of progressive pulmonary fibrosis and outcomes in connective tissue disease associated interstitial lung disease (CTD-ILD)

Jeewon Lee; Ahran  Kim; Kyuhwan Kim; Jung Hyun  Nam; Jong Min Lee; Yong Suk Jo

doi:10.36141/svdld.2026.18859

Authors

Jeewon Lee Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Ahran Kim Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, St. Vincent’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Kyuhwan Kim Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Jung Hyun Nam Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Jong Min Lee Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
Yong Suk Jo Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea

Keywords:

Connective Tissue Disease, Interstitial lung disease, Progressive Pulmonary Fibrosis

Abstract

Background and aim: Progressive pulmonary fibrosis (PPF) can develop in various interstitial lung diseases (ILD), including connective tissue disease-associated ILD (CTD-ILD). This study aimed to examine the longitudinal trajectory and transition of PPF status in patients with CTD-ILD.

Methods: In this single-center study at a tertiary referral hospital, we analyzed patients with CTD-ILD who underwent consecutive chest computed tomography (CT) scans and pulmonary function tests over two-year period. Patients were categorized into four groups based on annual PPF status.

Results: Among the 144 patients, the majority (n = 75) maintained a non-PPF status throughout follow-up (Group 1), while 25 patients developed PPF at year 2 (Group 2), 12 showed PPF improvement (Group 3), and 31 had persistent PPF (Group 4). The persistent PPF group showed significantly higher mortality risk (p = 0.007). Based on PPF status at 2 years, annual changes in forced vital capacity (FVC) and diffusing capacity of carbon monoxide (DLCO) were more pronounced in PPF groups than in non-PPF groups (FVC: -3.72 ± 0.52 %/year vs. 2.82 ± 0.41 %/year; DLCO: -4.30 ± 0.75 %/year vs. 3.64 ± 0.60 %/year). Patients treated with immunomodulating agents showed a reduced risk of progression to PPF, although the association was not statistically significant (Group 2, adjusted odds ratio: 0.26, 95% CI: 0.02-4.43, p = 0.351).

Conclusions: Regular pulmonary function monitoring and continuous assessment of PPF progression are essential for patients with CTD-ILD. Disease-directed treatment may reduce the risk of PPF development.

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