Clinical Features, Diagnosis, Management, and Outcomes of Idiopathic Pulmonary Fibrosis in Korea: Analysis of the Korea IPF Cohort (KICO) Registry
- Authors
- Man Pyo Chung, M.D., Ph.D.; Park, Jong Sun; Kim, Song Yee; Yoo, Hongseok; Song, Jin Woo; 정성환; Lee, Jae Ha; 이홍렬; Choi, Sun Mi; 김영환; Kim, Yong Hyun; 최혜숙; 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; 양세훈; 강형구; 제갈양진
- Issue Date
- Apr-2022
- Publisher
- 대한결핵및호흡기학회
- Keywords
- Idiopathic Pulmonary Fibrosis; Nationwide Registry; Antifibrotic Agent
- Citation
- Tuberculosis and Respiratory Diseases, v.85, no.2, pp.185 - 194
- Journal Title
- Tuberculosis and Respiratory Diseases
- Volume
- 85
- Number
- 2
- Start Page
- 185
- End Page
- 194
- URI
- https://scholarworks.bwise.kr/gachon/handle/2020.sw.gachon/84136
- DOI
- 10.4046/trd.2021.0123
- ISSN
- 1738-3536
- 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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