Tuberc Respir Dis.
2022 Apr;85(2):185-194. 10.4046/trd.2021.0123.
Clinical Features, Diagnosis, Management, and Outcomes of Idiopathic Pulmonary Fibrosis in Korea: Analysis of the Korea IPF Cohort (KICO) Registry
- Jegal Y
1 - Park JS2
- Kim SY3
- Yoo H4
- Jeong SH5
- Song JW6
- Lee JH7
- Lee HL8
- Choi SM9
- Kim YW10
- Kim YH11
- Choi HS12
- Lee J13
- Uh ST14
- Kim TH15
- Kim SH16
- Lee WY17
- Kim YH18
- Lee Hk19
- Lee EJ20
- Heo EY21
- Yang SH22
- Kang HK23
- Chung MP4
- on behalf of Korea ILD Study Group1
- Affiliations
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- 1Department of Internal Medicine, Ulsan University Hospital, University of Ulsan, College of Medicine, Ulsan, Korea
- 2Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea
- 3Division of Pulmonology, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- 4Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- 5Department of Internal Medicine, Gil Medical Center, Gachon Medical School, Incheon, Korea
- 6Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
- 7Division of Pulmonology, Department of Internal Medicine, Haeundae Paik Hospital, Inje University College of Medicine, Busan, Korea
- 8Department of Internal Medicine, Inha University School of Medicine, Incheon, Korea
- 9Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
- 10Departement of Respiratory-Allergy and Clinical Immunology, Konkuk University Hospital, Konkuk University School of Medicine, Seoul, Korea
- 11Division of Allergy and Pulmonology, Department of Internal Medicine, Bucheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Bucheon, Korea
- 12Department of Pulmonary and Critical Care Medicine, Kyung Hee Medical Center, Kyung Hee University School of Medicine, Seoul, Korea
- 13Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
- 14Division of Pulmonary and Allergy Medicine, Department of Internal Medicine, Soonchunhyang University Hospital, Seoul, Korea
- 15Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea
- 16Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University Hospital, Hanyang University College of Medicine, Seoul, Korea
- 17Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Internal Medicine, Yonsei University Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine, Wonju, Korea
- 18Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea
- 19Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
- 20Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Korea
- 21Division of Pulmonary and Critical Care, Department of Internal Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, Korea
- 22Department of Internal Medicine, Wonkwang University Hospital, Wonkwang University College of Medicine, Iksan, Korea
- 23Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea
- KMID: 2528422
- DOI: http://doi.org/10.4046/trd.2021.0123
Abstract
- Background
The Korea Interstitial Lung Disease Study Group has made a new nationwide idiopathic pulmonary fibrosis (IPF) registry because the routine clinical practice has changed due to new guidelines and newly developed antifibrotic agents in the recent decade. The aim of this study was to describe recent clinical characteristics of Korean IPF patients.
Methods
Both newly diagnosed and following IPF patients diagnosed after the previous registry in 2008 were enrolled. Survival analysis was only conducted for patients diagnosed with IPF after 2016 because antifibrotic agents started to be covered by medical insurance of Korea in October 2015.
Results
A total of 2,139 patients were analyzed. Their mean age at diagnosis was 67.4±9.3 years. Of these patients, 76.1% were males, 71.0% were ever-smokers, 14.4% were asymptomatic at the time of diagnosis, and 56.9% were at gender-agephysiology stage I. Occupational toxic material exposure was reported in 534 patients. The mean forced vital capacity was 74.6% and the diffusing capacity for carbon monoxide was 63.6%. Treatment with pirfenidone was increased over time: 62.4% of IPF patients were treated with pirfenidone initially. And 79.2% of patients were treated with antifiboritics for more than three months during the course of the disease since 2016. Old age, acute exacerbation, treatment without antifibrotics, and exposure to wood and stone dust were associated with higher mortality.
Conclusion
In the recent Korean IPF registry, the percentage of IPF patients treated with antifibrotics was increased compared to that in the previous IPF registry. Old age, acute exacerbation, treatment without antifibrotics, and exposure to wood and stone dust were associated with higher mortality.
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