ACUTE exacerbations (AEs) in patients with progressive pulmonary fibrosis (PPF) are both frequent and life-threatening, according to a new study published in Respiratory Research. Researchers have revealed that nearly one-third of PPF patients experience AEs, which substantially worsen survival outcomes, highlighting the urgent need for better management strategies.
The retrospective study examined clinical data from 133 patients diagnosed with PPF, excluding those with idiopathic pulmonary fibrosis (IPF). PPF was defined using the INBUILD trial criteria, and AEs were characterised by rapid-onset worsening of dyspnoea within 30 days, accompanied by new lung infiltrations and no evidence of heart failure. Over a median follow-up period of 38 months, 42 patients (31.6%) experienced AEs. The incidence rates were 12.5% at one year, 30.3% at three years, and 38.0% at five years.
Key risk factors for AEs included advanced age, rheumatoid arthritis-associated interstitial lung disease, fibrotic hypersensitivity pneumonitis, and reduced lung diffusing capacity for carbon monoxide (DLCO). Patients who experienced AEs had markedly poorer survival, with a median survival time of 30 months compared to an undefined survival time for those without AEs. The study found that AEs independently increased mortality risk by more than twofold (HR: 2.194, p = 0.004). Additional mortality predictors included older age, reduced DLCO, and the presence of a usual interstitial pneumonia-like pattern on high-resolution CT scans.
These findings showcase the impact of AEs on PPF progression and patient survival. The researchers stress the importance of early identification and management of high-risk patients to mitigate these outcomes. Developing targeted interventions to prevent AEs could improve survival rates and quality of life for those living with PPF.
Reference
Kim MJ et al. Acute exacerbation of progressive pulmonary fibrosis: incidence and outcomes. Respir Res. 2024;DOI: 10.1186/s12931-024-03048-x.
