{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T15:08:10Z","timestamp":1791385690861,"version":"4.3.3"},"reference-count":40,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,11,26]],"date-time":"2024-11-26T00:00:00Z","timestamp":1732579200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2024,11,26]],"date-time":"2024-11-26T00:00:00Z","timestamp":1732579200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea","doi-asserted-by":"publisher","award":["NRF-2022R1A2B5B02001602"],"award-info":[{"award-number":["NRF-2022R1A2B5B02001602"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Respir Res"],"DOI":"10.1186\/s12931-024-03048-x","type":"journal-article","created":{"date-parts":[[2024,11,26]],"date-time":"2024-11-26T04:17:54Z","timestamp":1732594674000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Acute exacerbation of progressive pulmonary fibrosis: incidence and outcomes"],"prefix":"10.1186","volume":"25","author":[{"given":"Min Jee","family":"Kim","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiyoul","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin Woo","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,11,26]]},"reference":[{"issue":"6","key":"3048_CR1","doi-asserted-by":"publisher","first-page":"733","DOI":"10.1164\/rccm.201308-1483ST","volume":"188","author":"WD Travis","year":"2013","unstructured":"Travis WD, Costabel U, Hansell DM, King TE Jr., Lynch DA, Nicholson AG, et al. An official American Thoracic Society\/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733\u201348.","journal-title":"Am J Respir Crit Care Med"},{"key":"3048_CR2","unstructured":"Annual Report on the Notified Tuberculosis in Korea. Cheongju, Korea: Korea Disease Control and Prevention Agency; 2021."},{"issue":"9","key":"3048_CR3","doi-asserted-by":"publisher","first-page":"e18","DOI":"10.1164\/rccm.202202-0399ST","volume":"205","author":"G Raghu","year":"2022","unstructured":"Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an Official ATS\/ERS\/JRS\/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e18\u201347.","journal-title":"Am J Respir Crit Care Med"},{"issue":"2","key":"3048_CR4","doi-asserted-by":"publisher","first-page":"356","DOI":"10.1183\/09031936.00159709","volume":"37","author":"JW Song","year":"2011","unstructured":"Song JW, Hong SB, Lim CM, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37(2):356\u201363.","journal-title":"Eur Respir J"},{"issue":"11","key":"3048_CR5","doi-asserted-by":"publisher","first-page":"2015","DOI":"10.1080\/03007995.2019.1647040","volume":"35","author":"M Wijsenbeek","year":"2019","unstructured":"Wijsenbeek M, Kreuter M, Olson A, Fischer A, Bendstrup E, Wells CD, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019;35(11):2015\u201324.","journal-title":"Curr Med Res Opin"},{"key":"3048_CR6","doi-asserted-by":"crossref","unstructured":"Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ. What\u2019s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5).","DOI":"10.1183\/13993003.00692-2018"},{"issue":"3","key":"3048_CR7","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1164\/rccm.201604-0801CI","volume":"194","author":"HR Collard","year":"2016","unstructured":"Collard HR, Ryerson CJ, Corte TJ, Jenkins G, Kondoh Y, Lederer DJ, et al. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. Am J Respir Crit Care Med. 2016;194(3):265\u201375.","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"3048_CR8","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1183\/09031936.06.00114004","volume":"27","author":"DS Kim","year":"2006","unstructured":"Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T. Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J. 2006;27(1):143\u201350.","journal-title":"Eur Respir J"},{"issue":"1","key":"3048_CR9","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1186\/s12931-021-01748-2","volume":"22","author":"J Kang","year":"2021","unstructured":"Kang J, Kim YJ, Choe J, Chae EJ, Song JW. Acute exacerbation of fibrotic hypersensitivity pneumonitis: incidence and outcomes. Respir Res. 2021;22(1):152.","journal-title":"Respir Res"},{"issue":"5","key":"3048_CR10","doi-asserted-by":"publisher","first-page":"525","DOI":"10.1111\/resp.13682","volume":"25","author":"A Suzuki","year":"2020","unstructured":"Suzuki A, Kondoh Y, Brown KK, Johkoh T, Kataoka K, Fukuoka J, et al. Acute exacerbations of fibrotic interstitial lung diseases. Respirology. 2020;25(5):525\u201334.","journal-title":"Respirology"},{"key":"3048_CR11","doi-asserted-by":"crossref","unstructured":"Kolb M, Bondue B, Pesci A, Miyazaki Y, Song JW, Bhatt NY et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150).","DOI":"10.1183\/16000617.0071-2018"},{"issue":"6","key":"3048_CR12","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1016\/j.rmed.2008.12.019","volume":"103","author":"T Suda","year":"2009","unstructured":"Suda T, Kaida Y, Nakamura Y, Enomoto N, Fujisawa T, Imokawa S, et al. Acute exacerbation of interstitial pneumonia associated with collagen vascular diseases. Respir Med. 2009;103(6):846\u201353.","journal-title":"Respir Med"},{"issue":"1","key":"3048_CR13","doi-asserted-by":"publisher","first-page":"282","DOI":"10.1186\/s12931-021-01879-6","volume":"22","author":"BS Kwon","year":"2021","unstructured":"Kwon BS, Choe J, Chae EJ, Hwang HS, Kim YG, Song JW. Progressive fibrosing interstitial lung disease: prevalence and clinical outcome. Respir Res. 2021;22(1):282.","journal-title":"Respir Res"},{"issue":"12","key":"3048_CR14","doi-asserted-by":"publisher","first-page":"1338","DOI":"10.1164\/rccm.200611-1685OC","volume":"177","author":"WD Travis","year":"2008","unstructured":"Travis WD, Hunninghake G, King TE Jr., Lynch DA, Colby TV, Galvin JR, et al. Idiopathic nonspecific interstitial pneumonia: report of an American thoracic society project. Am J Respir Crit Care Med. 2008;177(12):1338\u201347.","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"3048_CR15","doi-asserted-by":"publisher","first-page":"e36","DOI":"10.1164\/rccm.202005-2032ST","volume":"202","author":"G Raghu","year":"2020","unstructured":"Raghu G, Remy-Jardin M, Ryerson CJ, Myers JL, Kreuter M, Vasakova M, et al. Diagnosis of hypersensitivity pneumonitis in adults. An Official ATS\/JRS\/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202(3):e36\u201369.","journal-title":"Am J Respir Crit Care Med"},{"key":"3048_CR16","doi-asserted-by":"crossref","unstructured":"Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology\/European League against Rheumatism collaborative initiative. Ann Rheum Dis. 2010;69(9):1580\u20138.","DOI":"10.1136\/ard.2010.138461"},{"issue":"11","key":"3048_CR17","doi-asserted-by":"publisher","first-page":"2737","DOI":"10.1002\/art.38098","volume":"65","author":"F van den Hoogen","year":"2013","unstructured":"van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology\/European League against Rheumatism collaborative initiative. Arthritis Rheum. 2013;65(11):2737\u201347.","journal-title":"Arthritis Rheum"},{"issue":"1","key":"3048_CR18","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1136\/annrheumdis-2016-210571","volume":"76","author":"CH Shiboski","year":"2017","unstructured":"Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al. 2016 American College of Rheumatology\/European League Against Rheumatism classification criteria for primary Sj\u00f6gren\u2019s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis. 2017;76(1):9\u201316.","journal-title":"Ann Rheum Dis"},{"issue":"4","key":"3048_CR19","doi-asserted-by":"publisher","first-page":"285","DOI":"10.4046\/trd.2019.0009","volume":"82","author":"SM Koo","year":"2019","unstructured":"Koo SM, Kim SY, Choi SM, Lee HK. Korean guidelines for diagnosis and management of interstitial Lung diseases: Part 5. Connective tissue Disease Associated interstitial lung disease. Tuberc Respir Dis (Seoul). 2019;82(4):285\u201397.","journal-title":"Tuberc Respir Dis (Seoul)"},{"issue":"4","key":"3048_CR20","doi-asserted-by":"publisher","first-page":"720","DOI":"10.1183\/09031936.05.00034905","volume":"26","author":"N Macintyre","year":"2005","unstructured":"Macintyre N, Crapo RO, Viegi G, Johnson DC, van der Grinten CP, Brusasco V, et al. Standardisation of the single-breath determination of carbon monoxide uptake in the lung. Eur Respir J. 2005;26(4):720\u201335.","journal-title":"Eur Respir J"},{"issue":"2","key":"3048_CR21","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1183\/09031936.05.00034805","volume":"26","author":"MR Miller","year":"2005","unstructured":"Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, et al. Standardisation of spirometry. Eur Respir J. 2005;26(2):319\u201338.","journal-title":"Eur Respir J"},{"issue":"3","key":"3048_CR22","doi-asserted-by":"publisher","first-page":"511","DOI":"10.1183\/09031936.05.00035005","volume":"26","author":"J Wanger","year":"2005","unstructured":"Wanger J, Clausen JL, Coates A, Pedersen OF, Brusasco V, Burgos F, et al. Standardisation of the measurement of lung volumes. Eur Respir J. 2005;26(3):511\u201322.","journal-title":"Eur Respir J"},{"issue":"6","key":"3048_CR23","doi-asserted-by":"publisher","first-page":"1428","DOI":"10.1183\/09031936.00150314","volume":"44","author":"AE Holland","year":"2014","unstructured":"Holland AE, Spruit MA, Troosters T, Puhan MA, Pepin V, Saey D, et al. An official European Respiratory Society\/American Thoracic Society technical standard: field walking tests in chronic respiratory disease. Eur Respir J. 2014;44(6):1428\u201346.","journal-title":"Eur Respir J"},{"issue":"1","key":"3048_CR24","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1016\/j.chest.2022.01.007","volume":"162","author":"BS Kwon","year":"2022","unstructured":"Kwon BS, Lee HY, Choe J, Chae EJ, Hong S, Song JW. Acute Respiratory Deterioration in Rheumatoid Arthritis-Associated interstitial lung disease: a single-center study. Chest. 2022;162(1):136\u201344.","journal-title":"Chest"},{"issue":"38","key":"3048_CR25","doi-asserted-by":"publisher","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","volume":"43","author":"M Humbert","year":"2022","unstructured":"Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618\u2013731.","journal-title":"Eur Heart J"},{"issue":"18","key":"3048_CR26","doi-asserted-by":"publisher","first-page":"1718","DOI":"10.1056\/NEJMoa1908681","volume":"381","author":"KR Flaherty","year":"2019","unstructured":"Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. Nintedanib in Progressive Fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718\u201327.","journal-title":"N Engl J Med"},{"key":"3048_CR27","doi-asserted-by":"publisher","first-page":"175346662093577","DOI":"10.1177\/1753466620935774","volume":"14","author":"N Enomoto","year":"2020","unstructured":"Enomoto N, Naoi H, Aono Y, Katsumata M, Horiike Y, Yasui H, et al. Acute exacerbation of unclassifiable idiopathic interstitial pneumonia: comparison with idiopathic pulmonary fibrosis. Ther Adv Respir Dis. 2020;14:1753466620935774.","journal-title":"Ther Adv Respir Dis"},{"issue":"1","key":"3048_CR28","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1378\/chest.07-0323","volume":"132","author":"IN Park","year":"2007","unstructured":"Park IN, Kim DS, Shim TS, Lim CM, Lee SD, Koh Y, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007;132(1):214\u201320.","journal-title":"Chest"},{"issue":"9","key":"3048_CR29","doi-asserted-by":"publisher","first-page":"e003132","DOI":"10.1136\/bmjopen-2013-003132","volume":"3","author":"H Hozumi","year":"2013","unstructured":"Hozumi H, Nakamura Y, Johkoh T, Sumikawa H, Colby TV, Kono M, et al. Acute exacerbation in rheumatoid arthritis-associated interstitial lung disease: a retrospective case control study. BMJ Open. 2013;3(9):e003132.","journal-title":"BMJ Open"},{"issue":"2","key":"3048_CR30","doi-asserted-by":"publisher","first-page":"178","DOI":"10.4103\/atm.atm_14_21","volume":"16","author":"EH Alhamad","year":"2021","unstructured":"Alhamad EH, Cal JG, Alrajhi NN, AlBoukai AA. Acute exacerbation in interstitial lung disease. Ann Thorac Med. 2021;16(2):178\u201387.","journal-title":"Ann Thorac Med"},{"issue":"8","key":"3048_CR31","doi-asserted-by":"publisher","first-page":"1431","DOI":"10.1111\/resp.12862","volume":"21","author":"T Arai","year":"2016","unstructured":"Arai T, Kagawa T, Sasaki Y, Sugawara R, Sugimoto C, Tachibana K, et al. Heterogeneity of incidence and outcome of acute exacerbation in idiopathic interstitial pneumonia. Respirology. 2016;21(8):1431\u20137.","journal-title":"Respirology"},{"issue":"1","key":"3048_CR32","doi-asserted-by":"publisher","first-page":"215","DOI":"10.1186\/s12890-019-0960-1","volume":"19","author":"M Cao","year":"2019","unstructured":"Cao M, Sheng J, Qiu X, Wang D, Wang D, Wang Y, et al. Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study. BMC Pulm Med. 2019;19(1):215.","journal-title":"BMC Pulm Med"},{"issue":"7","key":"3048_CR33","doi-asserted-by":"publisher","first-page":"636","DOI":"10.1164\/rccm.200703-463PP","volume":"176","author":"HR Collard","year":"2007","unstructured":"Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King TE Jr., et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176(7):636\u201343.","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"3048_CR34","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1016\/j.rmed.2005.06.013","volume":"100","author":"K Jeon","year":"2006","unstructured":"Jeon K, Chung MP, Lee KS, Chung MJ, Han J, Koh WJ, et al. Prognostic factors and causes of death in Korean patients with idiopathic pulmonary fibrosis. Respir Med. 2006;100(3):451\u20137.","journal-title":"Respir Med"},{"issue":"7","key":"3048_CR35","doi-asserted-by":"publisher","first-page":"773","DOI":"10.1164\/rccm.201403-0566OC","volume":"190","author":"M Natsuizaka","year":"2014","unstructured":"Natsuizaka M, Chiba H, Kuronuma K, Otsuka M, Kudo K, Mori M, et al. Epidemiologic survey of Japanese patients with idiopathic pulmonary fibrosis and investigation of ethnic differences. Am J Respir Crit Care Med. 2014;190(7):773\u20139.","journal-title":"Am J Respir Crit Care Med"},{"issue":"5","key":"3048_CR36","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1016\/S2213-2600(20)30554-3","volume":"9","author":"J Behr","year":"2021","unstructured":"Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F, et al. Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial. Lancet Respir Med. 2021;9(5):476\u201386.","journal-title":"Lancet Respir Med"},{"issue":"1","key":"3048_CR37","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1186\/s12890-017-0462-y","volume":"17","author":"J Behr","year":"2017","unstructured":"Behr J, Neuser P, Prasse A, Kreuter M, Rabe K, Schade-Brittinger C, et al. Exploring efficacy and safety of oral pirfenidone for progressive, non-IPF lung fibrosis (RELIEF) - a randomized, double-blind, placebo-controlled, parallel group, multi-center, phase II trial. BMC Pulm Med. 2017;17(1):122.","journal-title":"BMC Pulm Med"},{"key":"3048_CR38","doi-asserted-by":"crossref","unstructured":"Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150).","DOI":"10.1183\/16000617.0076-2018"},{"issue":"9","key":"3048_CR39","doi-asserted-by":"publisher","first-page":"925","DOI":"10.1016\/S2213-2600(20)30355-6","volume":"8","author":"PM George","year":"2020","unstructured":"George PM, Spagnolo P, Kreuter M, Altinisik G, Bonifazi M, Martinez FJ, et al. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir Med. 2020;8(9):925\u201334.","journal-title":"Lancet Respir Med"},{"issue":"3","key":"3048_CR40","doi-asserted-by":"publisher","first-page":"750","DOI":"10.1183\/09031936.00131912","volume":"42","author":"CJ Ryerson","year":"2013","unstructured":"Ryerson CJ, Urbania TH, Richeldi L, Mooney JJ, Lee JS, Jones KD, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J. 2013;42(3):750\u20137.","journal-title":"Eur Respir J"}],"container-title":["Respiratory Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12931-024-03048-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s12931-024-03048-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s12931-024-03048-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,26]],"date-time":"2024-11-26T05:05:32Z","timestamp":1732597532000},"score":1,"resource":{"primary":{"URL":"https:\/\/respiratory-research.biomedcentral.com\/articles\/10.1186\/s12931-024-03048-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,26]]},"references-count":40,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3048"],"URL":"https:\/\/doi.org\/10.1186\/s12931-024-03048-x","relation":{},"ISSN":["1465-993X"],"issn-type":[{"value":"1465-993X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,11,26]]},"assertion":[{"value":"30 March 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 November 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 November 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The study protocol was approved by the Asan Medical Center Institutional Review Board (IRB No.: 2022\u2009\u2212\u20090116). Informed consent requirements were waived due to the retrospective nature of the study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"415"}}