ArticleRespiratory research2024
Acute exacerbation of progressive pulmonary fibrosis: incidence and outcomes.
Article in Respiratory research, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Cough burden in progressive pulmonary fibrosis: a systematic review with narrative synthesis.Health and quality of life outcomes · 2026Pooled it
- Can the Prognostic Nutritional Index Be a Predictor of Acute Exacerbation of Idiopathic Pulmonary Fibrosis?Biomedicines · 2026Article
- Construction and validation of an acute exacerbation risk score for fibrotic idiopathic interstitial pneumonia.Journal of thoracic disease · 2026Article
- Prevalence and Prognostic Impact of Progressive Pulmonary Fibrosis.Tuberculosis and respiratory diseases · 2026Review
- Acute Exacerbation of Interstitial Lung Disease: A Case Series and a Narrative Literature Review.Advances in respiratory medicine · 2026Review
- [58/m with progressive dyspnea, dry cough and general weakness : Preparation for the medical specialist examination: part 16].Medizinische Klinik, Intensivmedizin und Notfallmedizin · 2026Article
- UC-MSCs exhibit superior antifibrotic and anti-inflammatory effects compared to BM-MSCs in a bleomycin-induced idiopathic pulmonary fibrosis model.Open life sciences · 2026Article
- Microbiome-innate immune crosstalk in acute exacerbation of idiopathic pulmonary fibrosis: an amplification framework.Frontiers in immunology · 2026Review
- CONUT score as a prognostic biomarker in progressive pulmonary fibrosis: a simple tool for clinical risk stratification.BMC pulmonary medicine · 2025Article
- Oxymatrine attenuates pulmonary fibrosis via APE1‑mediated regulation of the PINK1/Parkin pathway.Molecular medicine reports · 2025Article
- Observational
- Pulmonary Hypertension-Related Interstitial Lung Disease: An Expert Opinion with a Real-World Approach.Biomedicines · 2025Article
- Baricitinib for Acute Exacerbation in Idiopathic Interstitial Pneumonias: A Preliminary Study Using Propensity Score Matching.Respiration; international review of thoracic diseases · 2025Article
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Authors and funding
3 authors.
Funding
Abstract
backgroundFew data are available on acute exacerbation (AE) in patients with progressive pulmonary fibrosis (PPF) besides idiopathic pulmonary fibrosis (IPF). This study aimed to investigate the AE incidence and outcomes among patients with PPF.
methodsClinical data of patients with PPF (n = 133) were retrospectively collected at a single center. PPF was defined based on the criteria used in the INBUILD trial. AE was defined as a worsening of dyspnea typically within 30 days with new bilateral lung infiltration and no evidence of cardiac failure or fluid overload.
resultsAmong patients with PPF, the mean age was 60.6 years old, 57.1% were females, and the most common etiology was connective tissue disease-related ILDs (63%). During the follow-up (median: 38.0 months) after PPF diagnosis, 42 patients (31.6%) experienced AE. The 1-, 3-, and 5-year AE incidences were 12.5%, 30.3%, and 38.0%, respectively. Older age, rheumatoid arthritis associated ILD, fibrotic hypersensitivity pneumonitis, and lower lung diffusing capacity for carbon monoxide were AE risk factors. Patients with AE demonstrated worse survival (median survival: 30 months vs. not reached; p < 0.001) after PPF diagnosis than those without. AE was independently associated with mortality in patients with PPF (hazard ratio [HR], 2.194; 95% confidence interval [CI], 1.285-3.747; p = 0.004) in the multivariable Cox analysis, along with older age, lower lung diffusing capacity for carbon monoxide, and the usual interstitial pneumonia-like pattern on high-resolution computed tomography.
conclusionsOur results suggest AE is not uncommon and significantly impacts on survival in patients with PPF.
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