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

  • Jegal, Yangjin (Department of Internal Medicine, Ulsan University Hospital, University of Ulsan, College of Medicine) ;
  • Park, Jong Sun (Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine) ;
  • Kim, Song Yee (Division of Pulmonology, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, Yonsei University College of Medicine) ;
  • Yoo, Hongseok (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jeong, Sung Hwan (Department of Internal Medicine, Gil Medical Center, Gachon Medical School) ;
  • Song, Jin Woo (Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Lee, Jae Ha (Division of Pulmonology, Department of Internal Medicine, Haeundae Paik Hospital, Inje University College of Medicine) ;
  • Lee, Hong Lyeol (Department of Internal Medicine, Inha University School of Medicine) ;
  • Choi, Sun Mi (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Kim, Young Whan (Departement of Respiratory-Allergy and Clinical Immunology, Konkuk University Hospital, Konkuk University School of Medicine) ;
  • Kim, Yong Hyun (Division of Allergy and Pulmonology, Department of Internal Medicine, Bucheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea) ;
  • Choi, Hye Sook (Department of Pulmonary and Critical Care Medicine, Kyung Hee Medical Center, Kyung Hee University School of Medicine) ;
  • Lee, Jongmin (Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea) ;
  • Uh, Soo-Taek (Division of Pulmonary and Allergy Medicine, Department of Internal Medicine, Soonchunhyang University Hospital) ;
  • Kim, Tae-Hyung (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine) ;
  • Kim, Sang-Heon (Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University Hospital, Hanyang University College of Medicine) ;
  • Lee, Won-Yeon (Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Internal Medicine, Yonsei University Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine) ;
  • Kim, Yee Hyung (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine) ;
  • Lee, Hyun-kyung (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Busan Paik Hospital, Inje University College of Medicine) ;
  • Lee, Eun Joo (Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University Anam Hospital, Korea University College of Medicine) ;
  • Heo, Eun Young (Division of Pulmonary and Critical Care, Department of Internal Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center) ;
  • Yang, Sei Hoon (Department of Internal Medicine, Wonkwang University Hospital, Wonkwang University College of Medicine) ;
  • Kang, Hyung Koo (Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine) ;
  • Chung, Man Pyo (Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Korea ILD Study Group, (Korea ILD Study Group)
  • 투고 : 2021.08.08
  • 심사 : 2021.12.13
  • 발행 : 2022.04.30

초록

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.

키워드

과제정보

The authors are grateful to all members of the Korean Interstitial Lung Disease Study Group, the pulmonologists, radiologists, and pathologists at the 23 hospitals who helped to gather data for analysis.

참고문헌

  1. Lee SH, Kim SY, Kim DS, Kim YW, Chung MP, Uh ST, et al. Predicting survival of patients with idiopathic pulmonary fibrosis using GAP score: a nationwide cohort study. Respir Res 2016;17:131. https://doi.org/10.1186/s12931-016-0454-0
  2. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014;370:2071-82. https://doi.org/10.1056/NEJMoa1402584
  3. King TE Jr, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014;370:2083-92. https://doi.org/10.1056/NEJMoa1402582
  4. Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, et al. Diagnosis of idiopathic pulmonary fibrosis: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2018;198:e44-68. https://doi.org/10.1164/rccm.201807-1255st
  5. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788-824. https://doi.org/10.1164/rccm.2009-040GL
  6. Costabel U, Albera C, Lancaster LH, Lin CY, Hormel P, Hulter HN, et al. An open-label study of the long-term safety of pirfenidone in patients with idiopathic pulmonary fibrosis (RECAP). Respiration 2017;94:408-15. https://doi.org/10.1159/000479976
  7. Behr J, Prasse A, Wirtz H, Koschel D, Pittrow D, Held M, et al. Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry. Eur Respir J 2020;56:1902279. https://doi.org/10.1183/13993003.02279-2019
  8. Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S, et al. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res 2018;19:141. https://doi.org/10.1186/s12931-018-0845-5
  9. Singh S, Collins BF, Sharma BB, Joshi JM, Talwar D, Katiyar S, et al. Interstitial lung disease in India. Results of a Prospective Registry. Am J Respir Crit Care Med 2017;195:801-13. https://doi.org/10.1164/rccm.201607-1484OC
  10. Wuyts WA, Dahlqvist C, Slabbynck H, Schlesser M, Gusbin N, Compere C, et al. Longitudinal clinical outcomes in a realworld population of patients with idiopathic pulmonary fibrosis: the PROOF registry. Respir Res 2019;20:231. https://doi.org/10.1186/s12931-019-1182-z
  11. Lee SH, Kim DS, Kim YW, Chung MP, Uh ST, Park CS, et al. Association between occupational dust exposure and prognosis of idiopathic pulmonary fibrosis: a Korean national survey. Chest 2015;147:465-74. https://doi.org/10.1378/chest.14-0994
  12. Kreuter M, Swigris J, Pittrow D, Geier S, Klotsche J, Prasse A, et al. The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry. Respir Res 2019;20:59. https://doi.org/10.1186/s12931-019-1020-3
  13. Jo HE, Glaspole I, Moodley Y, Chapman S, Ellis S, Goh N, et al. Disease progression in idiopathic pulmonary fibrosis with mild physiological impairment: analysis from the Australian IPF registry. BMC Pulm Med 2018;18:19. https://doi.org/10.1186/s12890-018-0575-y
  14. Snyder L, Neely ML, Hellkamp AS, O'Brien E, de Andrade J, Conoscenti CS, et al. Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry. Respir Res 2019;20:105. https://doi.org/10.1186/s12931-019-1043-9
  15. Salisbury ML, Conoscenti CS, Culver DA, Yow E, Neely ML, Bender S, et al. Antifibrotic drug use in patients with idiopathic pulmonary fibrosis: data from the IPF-PRO registry. Ann Am Thorac Soc 2020;17:1413-23. https://doi.org/10.1513/AnnalsATS.201912-880OC
  16. Fernandez-Fabrellas E, Molina-Molina M, Soriano JB, Portal JAR, Ancochea J, Valenzuela C, et al. Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry. Respir Res 2019;20:127. https://doi.org/10.1186/s12931-019-1084-0
  17. Lee SH, Park JS, Kim SY, Kim DS, Kim YW, Chung MP, et al. Comparison of CPI and GAP models in patients with idiopathic pulmonary fibrosis: a nationwide cohort study. Sci Rep 2018;8:4784. https://doi.org/10.1038/s41598-018-23073-3
  18. Jo HE, Glaspole I, Grainge C, Goh N, Hopkins PM, Moodley Y, et al. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J 2017;49:1601592. https://doi.org/10.1183/13993003.01592-2016
  19. Idiopathic Pulmonary Fibrosis Clinical Research Network; Raghu G, Anstrom KJ, King TE Jr, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012;366:1968-77. https://doi.org/10.1056/NEJMoa1113354
  20. Moon SW, Kim SY, Chung MP, Yoo H, Jeong SH, Kim DS, et al. Longitudinal changes in clinical features, management, and outcomes of idiopathic pulmonary fibrosis: a nationwide cohort study. Ann Am Thorac Soc 2021;18:780-7. https://doi.org/10.1513/AnnalsATS.202005-451OC