Predictors of Mortality in Progressive Fibrosing Interstitial Lung Diseases.

Xianqiu Chen,Ying Zhou,Jian Guo,Dong Yu,Bing Jie

doi:10.3389/fphar.2021.754851

Abstract

Background: Progressive fibrosing interstitial lung disease (PF-ILD) and idiopathic pulmonary fibrosis (IPF) share similar progression phenotype but with different pathophysiological mechanism. The purpose of this study was to assess clinical characteristics and outcomes of patients with PF-ILD in a single-center cohort. Methods: Patients with PF-ILD treated in Shanghai Pulmonary Hospital from Jan. 2013 to Dec. 2014 were retrospectively analyzed. Baseline characteristics and clinical outcomes were collected for survival analysis to identifying clinical predictors of mortality. Results: Among 608 patients with ILD, 132 patients met the diagnostic criteria for PF-ILD. In this single-center cohort, there were 51 (38.6%) cases with connective tissue disease-associated interstitial lung disease (CTD-ILD) and 45 (34.1%) with unclassifiable ILDs. During follow-up, 83 patients (62.9%) either died (N = 79, 59.8%) or underwent lung transplantations (N = 4, 3.0%) with a median duration follow-up time of 53.7 months. Kaplan-Meier survival curves revealed that the 1, 3 and 5-years survival of PF-ILD were 90.9, 58.8 and 48.1%, respectively. In addition, the prognosis of patients with PF-ILD was similar to those with IPF, while it was worse than non-PF-ILD ones. Multivariate Cox regression analysis demonstrated that high-resolution computed tomography (HRCT) scores (HR 1.684, 95% CI 1.017–2.788, p = 0.043) and systolic pulmonary artery pressure (SPAP) > 36.5 mmHg (HR 3.619, 95%CI 1.170–11.194, p = 0.026) were independent risk factors for the mortality of PF-ILD. Conclusion: Extent of fibrotic changes on HRCT and pulmonary hypertension were predictors of mortality in patients with PF-ILD.

Highlights

Progressive fibrosing interstitial lung disease (PF-ILD) is a terminology recently used to describe a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, and the underlying pathogenetic mechanism and clinical behaviors of which are similar to those of idiopathic pulmonary fibrosis (IPF) (Wells et al, 2018; Brown et al, 2020)
ILDs associated with a progressive fibrosing phenotype include non-specific interstitial pneumonia (NSIP) (Kim et al, 2010), unclassifiable idiopathic interstitial pneumonia (IIP) (Guler et al, 2018a), hypersensitivity pneumonitis (HP) (De Sadeleer et al, 2019), autoimmune ILDs (Doyle and Dellaripa, 2017; Guler et al, 2018b), sarcoidosis (Walsh et al, 2014a) and occupation-associated lung disease (Khalil et al, 2007)
Of the 608 patients with ILD seen over a 2-year period at Shanghai Pulmonary Hospital, 169 were identified as the progressive fibrosing phenotype

Summary

Introduction

Progressive fibrosing interstitial lung disease (PF-ILD) is a terminology recently used to describe a subset of patients who have inexorable progression of pulmonary fibrosis despite treatment, and the underlying pathogenetic mechanism and clinical behaviors of which are similar to those of idiopathic pulmonary fibrosis (IPF) (Wells et al, 2018; Brown et al, 2020). This single-center cohort study aims to identify risk factors from demographic information, clinical features, imaging data and blood biomarkers for mortality in Chinese PF-ILD population. Progressive fibrosing interstitial lung disease (PF-ILD) and idiopathic pulmonary fibrosis (IPF) share similar progression phenotype but with different pathophysiological mechanism. The purpose of this study was to assess clinical characteristics and outcomes of patients with PF-ILD in a single-center cohort

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