Exacerbation of idiopathic pulmonary fibrosis and connective tissue disease-related interstitial lung disease outcomes: a systematic review and meta-analysis

Introduction Acute exacerbations (AE) of idiopathic pulmonary fibrosis (IPF) and connective tissue disease-related interstitial lung disease (CTD-ILD) lack standardized treatments, and outcomes based on ILD etiology remain unclear. Whether adding immunosuppressive drugs to corticosteroids in IPF provides similar benefits as in CTD-ILD remains unknown. This study evaluates mortality and medical therapy effects on AE-IPF and AE-CTD-ILD outcomes.