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- Title
Progressive fibrosing interstitial lung disease: prevalence and clinical outcome.
- Authors
Kwon, Byoung Soo; Choe, Jooae; Chae, Eun Jin; Hwang, Hee Sang; Kim, Yong-Gil; Song, Jin Woo
- Abstract
Background: The progressive fibrosing (PF) phenotype of interstitial lung disease (ILD) is characterised by worsening respiratory symptoms, lung function, and extent of fibrosis on high-resolution computed tomography. We aimed to investigate the prevalence and clinical outcomes of PF-ILD in a real-world cohort and assess the prognostic significance of the PF-ILD diagnostic criteria. Methods: Clinical data of patients with fibrosing ILD other than idiopathic pulmonary fibrosis (IPF) consecutively diagnosed at a single centre were retrospectively reviewed. A PF phenotype was defined based on the criteria used in the INBUILD trial. Results: The median follow-up duration was 62.7 months. Of the total of 396 patients, the mean age was 58.1 years, 39.9% were men, and rheumatoid arthritis-ILD was the most common (42.4%). A PF phenotype was identified in 135 patients (34.1%). The PF-ILD group showed lower forced vital capacity and total lung capacity (TLC) than the non-PF-ILD group. The PF-ILD group also showed poorer survival (median survival, 91.2 months vs. not reached; P < 0.001) than the non-PF-ILD group. In multivariable Cox analysis adjusted for age, DLCO, HRCT pattern, and specific diagnosis, PF phenotype was independent prognostic factor (hazard ratio, 3.053; P < 0.001) in patients with fibrosing ILD. Each criterion of PF-ILD showed similar survival outcomes. Conclusions: Our results showed that approximately 34% of patients with non-IPF fibrosing ILD showed a progressive phenotype and a poor outcome similar to that of IPF, regardless of the diagnostic criteria used.
- Subjects
DISEASE prevalence; INTERSTITIAL lung diseases; IDIOPATHIC pulmonary fibrosis; VITAL capacity (Respiration); TREATMENT effectiveness; LUNG volume measurements
- Publication
Respiratory Research, 2021, Vol 22, Issue 1, p1
- ISSN
1465-9921
- Publication type
Article
- DOI
10.1186/s12931-021-01879-6