ArticleBMJ open respiratory research2023
Survival and acute exacerbation for patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias: 5-year follow-up analysis of a prospective multi-institutional patient registry.
Article in BMJ open respiratory research, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers, 3 of them syntheses that pooled it.
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Who cites it
31 citing papers in PubMed, 3 syntheses or guidelines pooled it, 30 citations in OpenAlex.
- Economic Burden of Idiopathic Pulmonary Fibrosis: Updated Evidence on Costs and Resource Use.PharmacoEconomics · 2026Pooled it
- Pooled it
- Serum surfactant protein D as a significant biomarker for predicting occurrence, progression, acute exacerbation, and mortality in interstitial lung disease: a systematic review and meta-analysis.Frontiers in immunology · 2025Pooled it
- Risk Model for Predicting Survival Outcomes Using Functional Exercise and Patient-Reported Outcomes in Fibrotic Interstitial Lung Disease: A Prospective Observational Study.Respirology (Carlton, Vic.) · 2025Trial
- Acute exacerbation in fibrotic interstitial lung disease: an international working group report.American journal of respiratory and critical care medicine · 2026Review
- Construction and validation of an acute exacerbation risk score for fibrotic idiopathic interstitial pneumonia.Journal of thoracic disease · 2026Article
- Acute Exacerbation of Interstitial Lung Disease: A Case Series and a Narrative Literature Review.Advances in respiratory medicine · 2026Review
- Three-minute walk test as a practical screening tool for detecting exertional hypoxemia in fibrotic interstitial lung disease: a real-world cohort study.Scientific reports · 2026Article
- Outcomes After Acute Respiratory Failure in Patients With Interstitial Lung Disease.CHEST pulmonary · 2026Article
- Prognostic Value of a Cardiopulmonary Exercise Testing-Derived Summed Score in Idiopathic Pulmonary Fibrosis and Connective Tissue Disease-Associated Interstitial Lung Disease: A Prospective Cohort Study.Respirology (Carlton, Vic.) · 2026Article
- Predictors of survival and transplantation outcomes in Japanese patients with interstitial lung disease referred for lung transplantation: a retrospective single-center study.BMC pulmonary medicine · 2026Article
- Article
- Interstitial Pneumonia with Autoimmune Features from the Rheumatologists' Perspective; Single Center Experience.Diagnostics (Basel, Switzerland) · 2026Article
- Association Between Single Nucleotide Polymorphisms and Disease Susceptibility, Survival, and Acute Exacerbation in Idiopathic Pulmonary Fibrosis.Respirology (Carlton, Vic.) · 2026Observational
- Interaction between NKG2D and its ligands MICA/B activates the DAP12/SYK/p53/p21 axis to drive pulmonary fibrosis.Frontiers in immunology · 2026Article
- Prognostic Impact of Radiologic and Pathologic Features on the Development of Progressive Pulmonary Fibrosis in Patients With Interstitial Lung Disease Other Than Idiopathic Pulmonary Fibrosis.Korean journal of radiology · 2026Article
- Evaluation of Factors Affecting Mortality in Patients with Idiopathic Pulmonary Fibrosis: A 10-Year Single-Center Experience.Diagnostics (Basel, Switzerland) · 2025Article
- Acute Exacerbation of Interstitial Lung Disease: Early Diagnosis and Treatment.Medicina (Kaunas, Lithuania) · 2025Review
- Diagnostic utility of high-mobility group box 1 for acute exacerbations of idiopathic pulmonary fibrosis.Scientific reports · 2025Observational
- Structure-based design of orthosteric and allosteric CCR2 inhibitors for potential IPF therapy.RSC advances · 2025Article
Corrections and comments
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Authors and funding
24 authors at 18 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveFew prospective cohort studies with relatively large numbers of patients with non-idiopathic pulmonary fibrosis (non-IPF) of idiopathic interstitial pneumonia (IIP) have been described. We aimed to assess disease progression and cause of death for patients with non-IPF IIPs or IPF under real-life conditions.
methodsData were analysed for a prospective multi-institutional cohort of 528 IIP patients enrolled in Japan between September 2013 and April 2016. Diagnosis of IPF versus non-IPF IIPs was based on central multidisciplinary discussion, and follow-up surveillance was performed for up to 5 years after patient registration. Survival and acute exacerbation (AE) were assessed.
resultsIPF was the most common diagnosis (58.0%), followed by unclassifiable IIPs (35.8%) and others (6.2%). The 5-year survival rate for non-IPF IIP and IPF groups was 72.8% and 53.7%, respectively, with chronic respiratory failure being the primary cause of death in both groups. AE was the second most common cause of death for both non-IPF IIP (24.1%) and IPF (23.5%) patients. The cumulative incidence of AE did not differ significantly between the two groups (p=0.36), with a 1-year incidence rate of 7.4% and 9.0% in non-IPF IIP and IPF patients, respectively. We found that 30.2% and 39.4% of non-IPF IIP and IPF patients, respectively, who experienced AE died within 3 months after an AE event, whereas 55.8% and 66.7% of such patients, respectively, died within 5 years after registration.
conclusionCloser monitoring of disease progression and palliative care interventions after AE are important for non-IPF IIP patients as well as for IPF patients.
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