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Prevalence, Clinical Features, and Outcomes of Young Patients with Idiopathic Pulmonary Fibrosis
Jeewon Lee1, Kyung Joo Kim2, Jung Hyun Nam2
1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea, jwleeca@gmail.com.
Insights
Younger idiopathic pulmonary fibrosis (IPF) patients face lower mortality risks but are less likely to receive antifibrotic therapy. Early, targeted treatments, including antifibrotics, are vital for improving outcomes in these patients.
Area of Science:
- Pulmonology
- Geriatric Medicine
- Clinical Research
Background:
- Idiopathic pulmonary fibrosis (IPF) primarily affects the elderly, with limited research on younger patient cohorts.
- Understanding the distinct clinical features and outcomes of younger IPF patients is crucial for tailored management.
Purpose of the Study:
- To investigate the clinical characteristics and outcomes of IPF patients under 50 years old.
- To compare younger IPF patients with older cohorts regarding disease progression and treatment responses.
Main Methods:
- Analysis of the Korean National Health Insurance Review and Assessment Service (HIRA) database (2015-2021).
- IPF patients identified via ICD-10 and Rare Intractable Diseases codes.
- Categorization into age groups: <50, 50-64, and ≥65 years.
- Analysis of risks for acute exacerbation (AE), mortality, and lung transplantation.
Main Results:
- Out of 4,243 IPF patients, 91 were under 50, predominantly female, with fewer comorbidities.
- Younger patients received more systemic steroids, while older patients received more pirfenidone.
- Mortality and lung transplantation risks significantly increased with age (HR: 1.52 for 50-64, HR: 2.44 for ≥65).
- Pirfenidone treatment was associated with reduced mortality risk.
Conclusions:
- Younger IPF patients exhibit lower mortality and lung transplantation risks but similar acute exacerbation risks compared to older patients.
- Underutilization of antifibrotic therapy and increased steroid use in younger IPF patients may impact outcomes.
- Implementing early, antifibrotic-focused treatment strategies is essential for improving the prognosis of young IPF patients.
Introduction:
Idiopathic pulmonary fibrosis (IPF) can occur at any age; however, studies on younger IPF patients are scarce because it primarily affects the elderly. This study aimed to investigate the clinical features and outcomes of younger IPF patients.
Methods:
We analyzed the National Korean Health Insurance Review and Assessment Service (HIRA) database from 2015 to 2021. Patients with IPF were identified using the International Classification of Diseases 10th Revision (ICD-10) codes and the Rare Intractable Diseases codes and were categorized into three age groups: <50, ≥50 and <65, and ≥65 years. The risk of acute exacerbation (AE) and mortality was analyzed.
Results:
Among 4,243 patients with IPF, 91 were under 50. These younger patients, who were predominantly female, exhibited less comorbidities and received more systemic steroids, whereas older group received more pirfenidone. Although AE risk increased with age, it was not statistically significant. Mortality and lung transplantation risks increased notably with age from the <50 group to the ≥50 and <65 group (hazard ratio [HR]: 1.52, 95% confidence interval [CI]: 0.93-2.49) and the ≥65 group (HR: 2.44, 95% CI: 1.51-3.93). These risks were influenced by factors such as age, comorbidities, previous AEs, and steroid use. Conversely, pirfenidone treatment reduced the risk.
Conclusion:
While younger IPF patients had a lower risk of mortality and lung transplantation, with no significant differences in the risk of AEs, they were less likely to receive antifibrotic therapy and more often treated with steroids, which may affect outcomes. Early, targeted treatment strategies, including antifibrotic use, are crucial for improving their prognosis.
Introduction:
Idiopathic pulmonary fibrosis (IPF) can occur at any age; however, studies on younger IPF patients are scarce because it primarily affects the elderly. This study aimed to investigate the clinical features and outcomes of younger IPF patients.
Methods:
We analyzed the National Korean Health Insurance Review and Assessment Service (HIRA) database from 2015 to 2021. Patients with IPF were identified using the International Classification of Diseases 10th Revision (ICD-10) codes and the Rare Intractable Diseases codes and were categorized into three age groups: <50, ≥50 and <65, and ≥65 years. The risk of acute exacerbation (AE) and mortality was analyzed.
Results:
Among 4,243 patients with IPF, 91 were under 50. These younger patients, who were predominantly female, exhibited less comorbidities and received more systemic steroids, whereas older group received more pirfenidone. Although AE risk increased with age, it was not statistically significant. Mortality and lung transplantation risks increased notably with age from the <50 group to the ≥50 and <65 group (hazard ratio [HR]: 1.52, 95% confidence interval [CI]: 0.93-2.49) and the ≥65 group (HR: 2.44, 95% CI: 1.51-3.93). These risks were influenced by factors such as age, comorbidities, previous AEs, and steroid use. Conversely, pirfenidone treatment reduced the risk.
Conclusion:
While younger IPF patients had a lower risk of mortality and lung transplantation, with no significant differences in the risk of AEs, they were less likely to receive antifibrotic therapy and more often treated with steroids, which may affect outcomes. Early, targeted treatment strategies, including antifibrotic use, are crucial for improving their prognosis.
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