Progressive fibrosing interstitial lung disease: prevalence and clinical outcome.

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 rheumatoi

SUBMITTER: Kwon BS 

PROVIDER: S-EPMC8559348 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature