Prognostication of progressive pulmonary fibrosis in connective tissue disease-associated interstitial lung diseases: A cohort study

Frontiers in Medicine

Medicine

Anne E. Wind¹  Mareye Voortman²  Jan C. Grutters³  Lucianne C. M. Langezaal⁴  H. Wouter van Es⁴  Maaike F. M. Koops⁵  Paco M. J. Welsing⁵  Julia Spierings⁵  Jacob M. van Laar⁵  Yu-Hsiang Chiu⁶  Anna Jamnitski⁷ 

[1] Department of Pulmonology, ILD Center of Excellence, St. Antonius Hospital, Nieuwegein, Netherlands;Department of Pulmonology, University Medical Center Utrecht, Utrecht, Netherlands;Department of Pulmonology, University Medical Center Utrecht, Utrecht, Netherlands;Department of Pulmonology, ILD Center of Excellence, St. Antonius Hospital, Nieuwegein, Netherlands;Department of Radiology, St. Antonius Hospital, Nieuwegein, Netherlands;Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands;Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, Netherlands;Division of Rheumatology, Immunology and Allergy, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan;Department of Rheumatology, St. Antonius Hospital, Nieuwegein, Netherlands;

关键词: interstitial lung diseases;    connective tissue diseases;    pulmonary fibrosis;    outcome predictors;    immune-mediated inflammatory diseases;   

DOI  :  10.3389/fmed.2023.1106560

 received in 2022-11-23, accepted in 2023-02-07,  发布年份 2023

来源: Frontiers

【 摘 要 】

BackgroundConnective tissue diseases-associated interstitial lung disease (CTD-ILD) is a heterogeneous condition that impairs quality of life and is associated with premature death. Progressive pulmonary fibrosis (PPF) has been identified as an important risk factor for poor prognosis. However, different criteria for PPF are used in clinical studies, which may complicate comparison between trials and translation of study findings into clinical practice.MethodsThis is a retrospective single center study in patients with CTD-ILD. The prognostic relevance of PPF definitions, including INBUILD, ATS/ERS/JRS/ALAT 2022, and simplified progressive fibrosing (simplified PF) criteria, were examined in this cohort and validated in the other reported Dutch CTD-ILD cohort.ResultsA total of 230 patients with CTD-ILD were included and the median follow-up period was six (3—9) years. Mortality risk was independently associated with age (adjusted HR 1.07, p < 0.001), smoking history (adjusted HR 1.90, p = 0.045), extent of fibrosis on high-resolution computed tomography (HRCT) at baseline (adjusted HR 1.05, p = 0.018) and baseline DLCO (adjusted HR 0.97, p = 0.013). Patients with regular pulmonary function tests in the first 2 years (adjusted HR 0.42, p = 0.002) had a better survival. The prognostic relevance for survival was similar between the three PPF criteria in the two cohorts.ConclusionHigher age, smoking, increased extent of fibrosis and low baseline DLCO were associated with poor prognosis, while regular pulmonary function evaluation was associated with better survival. The INBUILD, ATS/ERS/JRS/ALAT 2022, and simplified PF criteria revealed similar prognostication.

【 授权许可】

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Copyright © 2023 Chiu, Koops, Voortman, van Es, Langezaal, Welsing, Jamnitski, Wind, van Laar, Grutters and Spierings.