{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T18:48:23Z","timestamp":1792090103467,"version":"4.4.0"},"reference-count":40,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2019,8,19]],"date-time":"2019-08-19T00:00:00Z","timestamp":1566172800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2020,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background and objective<\/jats:title><jats:p>Acute exacerbation (AE) is a severe complication of idiopathic pulmonary fibrosis (AE\u2010IPF). In 2016, an international working group revised its definition and diagnostic criteria; however, few studies have assessed the frequency and prognosis of AE in patients with other fibrotic interstitial lung diseases (FILD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We used data from 1019 consecutive interstitial lung disease (ILD) patients initially evaluated between January 2008 and July 2015. All subject diagnoses were made by multidisciplinary discussion in December 2018. ILD was categorized as IPF (<jats:italic>n<\/jats:italic> =\u2009462) and other FILD which included non\u2010specific interstitial pneumonia (<jats:italic>n<\/jats:italic> =\u200922), chronic hypersensitivity pneumonitis (<jats:italic>n<\/jats:italic> =\u200929), connective tissue disease\u2010associated ILD (<jats:italic>n<\/jats:italic> =\u2009205) and unclassifiable ILD (<jats:italic>n<\/jats:italic> =\u2009209). Using the 2016 definition of AE\u2010IPF, we identified all subjects with an AE.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>During the observational period, 193 patients experienced a first AE (AE\u2010FILD <jats:italic>n<\/jats:italic> =\u200969, AE\u2010IPF <jats:italic>n<\/jats:italic> =\u2009124). The time to first AE was significantly longer in FILD than IPF (log\u2010rank test, <jats:italic>P<\/jats:italic> &lt;\u20090.001). After adjusting for potentially influential confounders, FILD remained a significant predictor of longer time to first AE compared with IPF (hazard ratio: 0.453; 95% CI: 0.317\u20130.647, <jats:italic>P<\/jats:italic> =\u20090.006). In a multivariate Cox proportional analysis, baseline disease severity was closely associated with the incidence of AE\u2010ILD. Even after adjustment for other clinical variables, AE had a negative impact on overall survival. AE\u2010FILD and AE\u2010IPF showed similar poor short\u2010term outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>All forms of ILD are at risk of AE and have a similar outcome to AE\u2010IPF.<\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.13682","type":"journal-article","created":{"date-parts":[[2019,8,19]],"date-time":"2019-08-19T23:58:38Z","timestamp":1566259118000},"page":"525-534","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":149,"title":["Acute exacerbations of fibrotic interstitial lung diseases"],"prefix":"10.1111","volume":"25","author":[{"given":"Atsushi","family":"Suzuki","sequence":"first","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Tosei General Hospital  Seto Japan"},{"name":"Department of Respiratory Medicine Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7456-5459","authenticated-orcid":false,"given":"Yasuhiro","family":"Kondoh","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Tosei General Hospital  Seto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin K.","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Medicine National Jewish Health  Denver CO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takeshi","family":"Johkoh","sequence":"additional","affiliation":[{"name":"Department of Radiology Kansai Rosai Hospital  Amagasaki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kensuke","family":"Kataoka","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Tosei General Hospital  Seto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junya","family":"Fukuoka","sequence":"additional","affiliation":[{"name":"Department of Pathology Nagasaki University Graduate School of Biomedical Sciences  Nagasaki Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomoki","family":"Kimura","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Tosei General Hospital  Seto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiaki","family":"Matsuda","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Tosei General Hospital  Seto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Toshiki","family":"Yokoyama","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine and Allergy Tosei General Hospital  Seto Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7645-9158","authenticated-orcid":false,"given":"Jun","family":"Fukihara","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahiko","family":"Ando","sequence":"additional","affiliation":[{"name":"Center for Advanced Medicine and Clinical Research Nagoya University Hospital  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonori","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Department of Pathology Kindai University Faculty of Medicine  Osakasayama Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naozumi","family":"Hashimoto","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Nagoya University Graduate School of Medicine  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshinori","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Nagoya University Graduate School of Medicine  Nagoya Japan"},{"name":"National Hospitalization Organization Nagoya Medical Center  Nagoya Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,8,19]]},"reference":[{"key":"e_1_2_8_2_1","unstructured":"RaghuG CollardHR EganJJ MartinezFJ BehrJ BrownKK ColbyTV CordierJF FlahertyKR LaskyJAet al.;ATS\/ERS\/JRS\/ALAT Committee on Idiopathic Pulmonary Fibrosis. 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