Abstract
BackgroundThe 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.MethodsClinical 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.ResultsThe 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.ConclusionsOur 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.
Highlights
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
A progressive phenotype was identified in 135 patients (34.1%)
progressive fibrosing ILD (PF-ILD) was the most frequently identified in fibrotic hypersensitivity pneumonitis (HP) (55.8%), followed by autoimmune ILD (32.1%) and interstitial pneumonia (iNSIP) (26.3%) (Fig. 2)
Summary
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 signifi‐ cance of the PF-ILD diagnostic criteria. Fibrosing ILDs other than IPF, such as idiopathic nonspecific interstitial pneumonia (iNSIP), fibrotic hypersensitivity pneumonitis (HP), and autoimmune ILD, have a progressive phenotype, manifesting as worsening. The proportion of a progressive phenotype in non-IPF ILDs was reported to be approximately 30% in a realworld [2, 10]. Data on PF-ILD are still limited, such as its proportion among non-IPF fibrosing ILD, risk factors, and clinical outcomes in a real-world cohort. The aim of this study was to evaluate the prevalence, risk factors, and survival of PF-ILD, and to assess the prognostic value of various PF-ILD criteria in patients with non-IPF fibrosing ILD
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