| Nationwide Incidence and Prevalence of Idiopathic Pulmonary Fibrosis in South Korea |
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Hee-Young Yoon1, Kyung Hoon Kim2, Song Yee Kim3, Moo Suk Park3, Jong Sun Park4, Joo Hun Park5, Soo Taek Uh1, Jung-Wan Yoo6, Jin Hwa Lee7, Won-Il Choi8, Jae Ha Lee9, Chang Hoon Han10, on behalf of Korea ILD Study Group |
1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Soonchunhyang University Seoul Hospital, Seoul, Republic of Korea
2Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Incheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea
3Division of Pulmonology, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
4Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
5Department of Pulmonology and Critical Care Medicine, Ajou University School of Medicine, Suwon, Republic of Korea
6Department of Internal Medicine, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Republic of Korea
7Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea
8Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Myongji Hospital, Goyang, Republic of Korea
9Division of Pulmonology, Department of Internal Medicine, Inje University Haeundae Paik Hospital, Inje University College of Medicine, Busan, Republic of Korea
10Division of Pulmonology, Department of Internal Medicine, National Health Insurance Service Ilsan Hospital, Goyang, Republic of Korea |
Correspondence:
Jae Ha Lee, Tel: +82-51-797-0457, Fax: +82-51-797-2210,
Email: anilleus@naver.com
Chang Hoon Han, Tel: +82-31-900-0008, Fax: +82-31-900-0343,
Email: chang122@nhimc.or.kr
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Received: 15 January 2026 • Revised: 27 March 2026 • Accepted: 18 June 2026 |
| Abstract |
Background
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with high mortality. Population-based data on long-term epidemiological trends and real-world treatment outcomes in Asia remain limited. This study described national trends in IPF prevalence and incidence in South Korea and evaluated pirfenidone use and associated mortality.
Methods
Using the Korean National Health Insurance Service customized research database, we identified prevalent IPF cases from 2010 to 2023 and incident cases from 2011 to 2023. IPF was defined using the International Classification of Diseases, 10th Revision, and rare incurable disease codes, based on a predefined operational definition. Among incident cases, the association between pirfenidone use and all-cause mortality was assessed using multivariable-adjusted Cox proportional hazards models.
Results
A total of 91,638 prevalent and 28,683 incident IPF cases were identified during the study period. The crude prevalence increased from 8.91 per 100,000 persons ≥50 years in 2010 to 63.78 per 100,000 in 2023. The crude incidence increased from 6.07 per 100,000 in 2011 to 16.86 per 100,000 in 2023. Similar increasing patterns were observed for age- and sex-standardized rates. IPF was more common in men, and demonstrated a pronounced age gradient. Following reimbursement approval, pirfenidone use increased substantially. Pirfenidone use was associated with a reduced risk of all-cause mortality (adjusted hazard ratio, 0.627; 95% confidence interval, 0.600–0.665), with progressively stronger associations observed with longer treatment duration.
Conclusion
The burden of IPF in South Korea has increased over the past decade, during which expanded real-world pirfenidone use was associated with improved survival. |
| Key Words:
Pulmonary Fibrosis, Idiopathic, Epidemiology, Incidence, Prevalence, Pirfenidone |
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