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KMID : 1044520220850020185
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Tuberculosis and Respiratory Diseases
2022 Volume.85 No. 2 p.185 ~ p.194
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Clinical Features, Diagnosis, Management, and Outcomes of Idiopathic Pulmonary Fibrosis in Korea: Analysis of the Korea IPF Cohort (KICO) Registry
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Jegal Yang-Jin
Park Jong-Sun
Kim Song-Yee
Yoo Hong-Seok
Jeong Sung-Hwan
Song Jin-Woo
Lee Jae-Ha
Lee Hong-Lyeol
Choi Sun-Mi
Kim Young-Whan
Kim Yong-Hyun
Choi Hye-Sook
Lee Jong-Min
Uh Soo-Taek
Kim Tae-Hyung
Kim Sang-Heon
Lee Won-Yeon
Kim Yee-Hyung
Lee Hyun-Kyung
Lee Eun-Joo
Heo Eun-Young
Yang Sei-Hoon
Kang Hyung-Koo
Chung Man-Pyo
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Abstract
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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-age-physiology 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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KEYWORD
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Idiopathic Pulmonary Fibrosis, Nationwide Registry, Antifibrotic Agent
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