Longitudinal Changes in Clinical Features, Management, and Outcomes of Idiopathic Pulmonary Fibrosis. A Nationwide Cohort Study.

Sung Woo Moon, Song Yee Kim, Man Pyo Chung, Hongseok Yoo, Sung Hwan Jeong, Dong Soon Kim, Jin Woo Song, Hong Lyeol Lee, Sun Mi Choi, Young Whan Kim, Yong Hyun Kim, Choon-Sik Park, Sung-Woo Park, Jong Sun Park, Yangjin Jegal, Jongmin Lee, Soo-Taek Uh, Tae-Hyung Kim, Jae Ha Lee, Yee Hyung Kim, Bumsu Shin, Hyun-Kyung Lee, Sei-Hoon Yang, Hyun Lee, Sang-Heon Kim, Eun-Joo Lee, Hye Sook Choi, Hyejung Shin, Yong Bum Park, Jong Wook Shin, Moo Suk Park

Journal: Annals of the American Thoracic Society 2021;18(5):780-787

PMID: 33270528

Full Text on Atypon View on PubMed

Abstract

In recent decades, diagnosis and treatment recommendations for idiopathic pulmonary fibrosis (IPF) have changed. In Korea, the average life expectancy has increased, unmet healthcare needs have been reduced, and the number of computed tomographic examinations performed has nearly doubled. The Korean Interstitial Lung Disease Study Group conducted a nationwide cohort study for idiopathic interstitial pneumonia, including IPF, and established a registry for IPF. Using study data collected by the study group, this study aimed to evaluate longitudinal changes in clinical features, diagnosis, treatment, and mortality and analyze the extent to which changes in medication usage affected IPF-associated mortality. The study population included newly diagnosed patients with IPF from a cohort study (January 2002 to September 2008, = 1,839, 2008 group) and prospective registry (January 2012 to August 2018, = 1,345, 2018 group). Survival curves were estimated using the Kaplan-Meier method, and Cox regression models were used to identify mortality-associated risk factors in each group. The 2018 group was younger, had fewer symptoms, had less honeycombing, underwent more serologic autoimmune marker and pulmonary function tests, had higher oxygen partial pressure and lower carbon dioxide partial pressure values, was less frequently diagnosed by surgical biopsy, and had better survival than the 2008 group. Steroid use and conservative care declined, whereas -acetylcysteine use increased in this group. Antifibrotic agents were used in only the 2018 group. In the 2008 group, -acetylcysteine was associated with lower mortality, whereas conservative care was associated with higher mortality. In the 2018 group, the use of antifibrotic agents was associated with lower mortality, and steroid use was associated with higher mortality. The survival rates in the 2008 and 2018 non-antifibrotic agent subgroups were similar. This study analyzed national IPF cohort data spanning 17 years. In clinical practice, the IPF diagnosis was made earlier, steroid and immunosuppressive agent use was reduced, and antifibrotic agents were administered. The survival of patients with IPF has improved over the decades, and antifibrotic use was consistently associated with improved survival.Clinical trial registered with clinicaltrials.gov (NCT04160715).

Address: Division of Pulmonology, Department of Internal Medicine, Institute of Chest Diseases, Severance Hospital, and.; Division of Pulmonary and Critical Care Medicine, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul, South Korea.; Department of Internal Medicine, Gil Medical Center, Gachon Medical School, Incheon, South Korea.; Division of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, South Korea.; Department of Internal Medicine, School of Medicine, Inha University, Incheon, South Korea.; Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, College of Medicine, Seoul National University, Seoul, South Korea.; Division of Allergy and Pulmonology, Department of Internal Medicine, Bucheon St. Mary's Hospital, School of Medicine, The Catholic University of Korea, Bucheon-si, South Korea.; Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon-si, South Korea.; Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam-si, South Korea.; Division of PulmonaryMedicine, Department of Internal Medicine, Ulsan University Hospital, College of Medicine, University of Ulsan, Ulsan, South Korea.; 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, Seoul, South Korea.; Division of Pulmonary and Allergy Medicine, Department of Internal Medicine, Soonchunhyang University Hospital, Seoul, South Korea.; Division of Pulmonary and Critical Care Medicine, Hanyang University Guri Hospital, College of Medicine, Hanyang University, Guri, South Korea.; Division of Pulmonology, Department of Internal Medicine, Haeundae Paik Hospital, College of Medicine, Inje University, Busan, South Korea.; Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kyung Hee University Hospital at Gangdong, and.; Department of Internal Medicine, Wonju College of Medicine, Yonsei University, Wonju, South Korea.; Department of Internal Medicine, Busan Paik Hospital, Inje University, Busan, South Korea.; Division of Pulmonary, Department of Internal Medicine, College of Medicine, Wonkwang University, Iksan, South Korea.; Department of Internal Medicine, College of Medicine, Hanyang University, Seoul, South Korea.; Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University Anam Hospital, College of Medicine, Korea University, Seoul, South Korea.; Department of Pulmonary and Critical Care Medicine, Kyung Hee Medical Center, School of Medicine, Kyung Hee University, Seoul, South Korea.; Biostatistics Collaboration Unit, Department of Biomedical Systems Informatics, College of Medicine, Yonsei University, Seoul, South Korea.; Department of Internal Medicine, Division of Pulmonary, Allergy and Critical Care Medicine, Kangdong Sacred Heart Hospital, Hallym University, Seoul, South Korea; and.; Division of Pulmonary Medicine, Department of Internal Medicine, College of Medicine, Chung Ang University, Seoul, South Korea.

DOI: 10.1513/AnnalsATS.202005-451OC

Link outs

Free resources

Medical:

Subscription / membership required

Full Text Sources:

MeSH Terms: Biopsy, Cohort Studies, Humans, Idiopathic Pulmonary Fibrosis, Lung Diseases, Interstitial, Respiratory Function Tests