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Clinical Features, Diagnosis, Management and Outcomes of Idiopathic Pulmonary Fibrosis in Korea: Analysis of the Korea IPF Cohort (KICO) Registry

Cited 14 time in Web of Science Cited 13 time in Scopus
Authors

Jegal, Yangjin; Park, Jong Sun; Kim, Song Yee; Yoo, Hongseok; 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, Jongmin; 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

Issue Date
2022-04
Publisher
대한결핵및호흡기학회
Citation
Tuberculosis and Respiratory Diseases, Vol.85 No.2, pp.185-194
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-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.
ISSN
1738-3536
URI
https://hdl.handle.net/10371/183693
DOI
https://doi.org/10.4046/trd.2021.0123
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