{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T07:53:40Z","timestamp":1790754820481,"version":"4.1.0"},"reference-count":103,"publisher":"European Respiratory Society (ERS)","issue":"145","content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir Rev"],"accepted":{"date-parts":[[2017,7,4]]},"published-print":{"date-parts":[[2017,9,30]]},"abstract":"<jats:p>Recognising recent advances, the definition and diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) have been updated by an international working group. The new definition describes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events) characterised by evidence of new widespread alveolar abnormality. The new criteria require a previous or concurrent diagnosis of IPF, an acute worsening or development of dyspnoea typically less than 1\u2005month in duration, chest imaging evidence on computed tomography (CT) of new bilateral ground-glass opacity and\/or consolidation superimposed on a background imaging pattern of usual interstitial pneumonia not fully explained by cardiac failure or fluid overload. Due to high in-hospital mortality rates, current treatment guidelines say that the majority of patients with AE-IPF should not receive mechanical ventilation. However, new data suggest that the prognosis may have improved. This modest improvement in overall survival seen in more recent studies may be the result of differences in the diagnostic criteria, study design, baseline clinical risk factors and\/or improvements in management. Based on our updated knowledge of possible preventive and therapeutic measures, including mechanical ventilation and pharmacological therapies, the current approach to the treatment of AE-IPF requires careful decision-making.<\/jats:p>","DOI":"10.1183\/16000617.0050-2017","type":"journal-article","created":{"date-parts":[[2017,9,28]],"date-time":"2017-09-28T00:10:25Z","timestamp":1506557425000},"page":"170050","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":66,"title":["Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis"],"prefix":"10.1183","volume":"26","author":[{"given":"Yasuhiro","family":"Kondoh","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin K.","family":"Brown","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2017,9,27]]},"reference":[{"key":"2024102403294362000_26.145.170050.1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2009-040GL"},{"key":"2024102403294362000_26.145.170050.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)60052-4"},{"key":"2024102403294362000_26.145.170050.3","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200703-463PP"},{"key":"2024102403294362000_26.145.170050.4","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201604-0801CI"},{"key":"2024102403294362000_26.145.170050.5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1843.2010.01774.x"},{"key":"2024102403294362000_26.145.170050.6","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00159709"},{"key":"2024102403294362000_26.145.170050.7","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-14-73"},{"key":"2024102403294362000_26.145.170050.8","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1111\/resp.12786","article-title":"Acute and subacute idiopathic interstitial pneumonias","volume":"21","author":"Taniguchi","year":"2016","journal-title":"Respirology"},{"key":"2024102403294362000_26.145.170050.9","first-page":"359","article-title":"Clinical analysis of the acute exacerbation in patients with idiopathic pulmonary fibrosis","volume":"44","author":"Okamoto","year":"2006","journal-title":"Nihon Kokyuki Gakkai Zasshi"},{"key":"2024102403294362000_26.145.170050.10","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2005.06.013"},{"key":"2024102403294362000_26.145.170050.11","first-page":"103","article-title":"Risk factors of acute exacerbation of idiopathic pulmonary fibrosis","volume":"27","author":"Kondoh","year":"2010","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"2024102403294362000_26.145.170050.12","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201006-0894CI"},{"key":"2024102403294362000_26.145.170050.13","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201403-0566OC"},{"key":"2024102403294362000_26.145.170050.14","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2008.06.003"},{"key":"2024102403294362000_26.145.170050.15","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.168.1.8976924"},{"key":"2024102403294362000_26.145.170050.16","doi-asserted-by":"publisher","DOI":"10.1378\/chest.124.4.1206"},{"key":"2024102403294362000_26.145.170050.17","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.42.565"},{"key":"2024102403294362000_26.145.170050.18","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1155\/2004\/379723","article-title":"Outcome of patients admitted to the intensive care unit for acute exacerbation of idiopathic pulmonary fibrosis","volume":"11","author":"Al-Hameed","year":"2004","journal-title":"Can Respir J"},{"key":"2024102403294362000_26.145.170050.19","doi-asserted-by":"publisher","DOI":"10.1378\/chest.128.3.1475"},{"key":"2024102403294362000_26.145.170050.20","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.06.00114004"},{"key":"2024102403294362000_26.145.170050.21","doi-asserted-by":"publisher","DOI":"10.1097\/01.rti.0000213588.52343.13"},{"key":"2024102403294362000_26.145.170050.22","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1843.2007.01119.x"},{"key":"2024102403294362000_26.145.170050.23","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200709-1365OC"},{"key":"2024102403294362000_26.145.170050.24","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.90637.2008"},{"key":"2024102403294362000_26.145.170050.25","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2010.137190"},{"key":"2024102403294362000_26.145.170050.26","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.50.4327"},{"key":"2024102403294362000_26.145.170050.27","doi-asserted-by":"publisher","DOI":"10.1159\/000330325"},{"key":"2024102403294362000_26.145.170050.28","first-page":"113","article-title":"Polymyxin-B hemoperfusion for acute exacerbation of idiopathic pulmonary fibrosis: serum IL-7 as a prognostic marker","volume":"28","author":"Tachibana","year":"2011","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"2024102403294362000_26.145.170050.29","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-011-2211-6"},{"key":"2024102403294362000_26.145.170050.30","doi-asserted-by":"publisher","DOI":"10.1159\/000329891"},{"key":"2024102403294362000_26.145.170050.31","doi-asserted-by":"publisher","DOI":"10.1159\/000329893"},{"key":"2024102403294362000_26.145.170050.32","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00050911"},{"key":"2024102403294362000_26.145.170050.33","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00106011"},{"key":"2024102403294362000_26.145.170050.34","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.51.6965"},{"key":"2024102403294362000_26.145.170050.35","first-page":"9","article-title":"Clinical characteristics of acute exacerbations of idiopathic pulmonary fibrosis and involvement of viral, Mycoplasma pneumoniae, and Chlamydophila pneumoniae infections","volume":"40","author":"Anzai","year":"2013","journal-title":"Dokkyo J Med Sci"},{"key":"2024102403294362000_26.145.170050.36","doi-asserted-by":"crossref","first-page":"903","DOI":"10.7150\/ijms.4972","article-title":"Procalcitonin-guided antibiotic use in acute exacerbations of idiopathic pulmonary fibrosis","volume":"10","author":"Ding","year":"2013","journal-title":"Int J Med Sci"},{"key":"2024102403294362000_26.145.170050.37","doi-asserted-by":"publisher","DOI":"10.1007\/s12192-013-0411-5"},{"key":"2024102403294362000_26.145.170050.38","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00122213"},{"key":"2024102403294362000_26.145.170050.39","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201203-0506OC"},{"key":"2024102403294362000_26.145.170050.40","doi-asserted-by":"crossref","unstructured":"Usui Y , Kaga A , Sakai F , et al. A cohort study of mortality predictors in patients with acute exacerbation of chronic fibrosing interstitial pneumonia. BMJ Open 2013; 3: e002971.","DOI":"10.1136\/bmjopen-2013-002971"},{"key":"2024102403294362000_26.145.170050.41","doi-asserted-by":"publisher","DOI":"10.1007\/s00408-013-9530-0"},{"key":"2024102403294362000_26.145.170050.42","first-page":"121","article-title":"The effect of anticoagulant therapy for idiopathic pulmonary fibrosis in real life practice","volume":"30","author":"Tomassetti","year":"2013","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"2024102403294362000_26.145.170050.43","doi-asserted-by":"publisher","DOI":"10.1016\/j.pupt.2014.04.008"},{"key":"2024102403294362000_26.145.170050.44","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.49.3222"},{"key":"2024102403294362000_26.145.170050.45","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-1481"},{"key":"2024102403294362000_26.145.170050.46","doi-asserted-by":"publisher","DOI":"10.1159\/000225932"},{"key":"2024102403294362000_26.145.170050.47","doi-asserted-by":"publisher","DOI":"10.4187\/respcare.01734"},{"key":"2024102403294362000_26.145.170050.48","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2013.06.039"},{"key":"2024102403294362000_26.145.170050.49","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcrc.2014.03.019"},{"key":"2024102403294362000_26.145.170050.50","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.rmed.2015.12.005","article-title":"The use of mechanical ventilation in patients with idiopathic pulmonary fibrosis in the United States: A nationwide retrospective cohort analysis","volume":"111","author":"Rush","year":"2016","journal-title":"Respir Med"},{"key":"2024102403294362000_26.145.170050.51","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1186\/s12890-016-0253-x","article-title":"Efficacy of concurrent treatments in idiopathic pulmonary fibrosis patients with a rapid progression of respiratory failure: an analysis of a national administrative database in Japan","volume":"16","author":"Oda","year":"2016","journal-title":"BMC Pulm Med"},{"key":"2024102403294362000_26.145.170050.52","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200404-571OC"},{"key":"2024102403294362000_26.145.170050.53","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200705-732OC"},{"key":"2024102403294362000_26.145.170050.54","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(09)60551-1"},{"key":"2024102403294362000_26.145.170050.55","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00005209"},{"key":"2024102403294362000_26.145.170050.56","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1002110"},{"key":"2024102403294362000_26.145.170050.57","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201011-1874OC"},{"key":"2024102403294362000_26.145.170050.58","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1103690"},{"key":"2024102403294362000_26.145.170050.59","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(11)60405-4"},{"key":"2024102403294362000_26.145.170050.60","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201202-0314OC"},{"key":"2024102403294362000_26.145.170050.61","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1402584"},{"key":"2024102403294362000_26.145.170050.62","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1401739"},{"key":"2024102403294362000_26.145.170050.63","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0166462"},{"key":"2024102403294362000_26.145.170050.64","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201011-1790OC"},{"key":"2024102403294362000_26.145.170050.65","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1378\/chest.13-2424","article-title":"Outcomes after hospitalization in idiopathic pulmonary fibrosis: a cohort study","volume":"147","author":"Brown","year":"2015","journal-title":"Chest"},{"key":"2024102403294362000_26.145.170050.66","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1016\/j.chest.2015.12.026","article-title":"Patients with fibrotic interstitial lung disease hospitalized for acute respiratory worsening: a large cohort analysis","volume":"149","author":"Moua","year":"2016","journal-title":"Chest"},{"key":"2024102403294362000_26.145.170050.67","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/s12890-015-0004-4","article-title":"Treatment of acute exacerbation of idiopathic pulmonary fibrosis with direct hemoperfusion using a polymyxin B-immobilized fiber column improves survival","volume":"15","author":"Enomoto","year":"2015","journal-title":"BMC Pulm Med"},{"key":"2024102403294362000_26.145.170050.68","doi-asserted-by":"publisher","DOI":"10.1378\/chest.14-2746"},{"key":"2024102403294362000_26.145.170050.69","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201503-0562OC"},{"key":"2024102403294362000_26.145.170050.70","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200810-1534OC"},{"key":"2024102403294362000_26.145.170050.71","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1007\/s13665-013-0065-x","article-title":"Acute exacerbation of idiopathic pulmonary fibrosis: a proposal","volume":"2","author":"Johannson","year":"2013","journal-title":"Curr Respir Care Rep"},{"key":"2024102403294362000_26.145.170050.72","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1159\/000468546","article-title":"Antacid therapy and disease progression in patients with idiopathic pulmonary fibrosis who received pirfenidone","volume":"93","author":"Kreuter","year":"2017","journal-title":"Respiration"},{"key":"2024102403294362000_26.145.170050.73","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1503326"},{"key":"2024102403294362000_26.145.170050.74","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2006.02.002"},{"key":"2024102403294362000_26.145.170050.75","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1007\/s12325-015-0249-6","article-title":"Development of a consensus statement for the definition, diagnosis, and treatment of acute exacerbations of idiopathic pulmonary fibrosis using the Delphi technique","volume":"32","author":"Maher","year":"2015","journal-title":"Adv Ther"},{"key":"2024102403294362000_26.145.170050.76","doi-asserted-by":"publisher","DOI":"10.1007\/s11748-014-0487-6"},{"key":"2024102403294362000_26.145.170050.77","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2016.02.001"},{"key":"2024102403294362000_26.145.170050.78","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1402582"},{"key":"2024102403294362000_26.145.170050.79","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1186\/s12931-016-0398-4","article-title":"A phase II trial evaluating the efficacy and safety of perioperative pirfenidone for prevention of acute exacerbation of idiopathic pulmonary fibrosis in lung cancer patients undergoing pulmonary resection: West Japan Oncology Group 6711 L (PEOPLE Study)","volume":"17","author":"Iwata","year":"2016","journal-title":"Respir Res"},{"key":"2024102403294362000_26.145.170050.80","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.42.565"},{"key":"2024102403294362000_26.145.170050.81","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.44.1144"},{"key":"2024102403294362000_26.145.170050.82","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.49.2359"},{"key":"2024102403294362000_26.145.170050.83","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.50.4327"},{"key":"2024102403294362000_26.145.170050.84","doi-asserted-by":"crossref","first-page":"478","DOI":"10.1159\/000358443","article-title":"Efficacy of azithromycin for treatment of acute exacerbation of chronic fibrosing interstitial pneumonia: a prospective, open-label study with historical controls","volume":"87","author":"Kawamura","year":"2014","journal-title":"Respiration"},{"key":"2024102403294362000_26.145.170050.85","doi-asserted-by":"crossref","first-page":"903","DOI":"10.7150\/ijms.4972","article-title":"Procalcitonin-guided antibiotic use in acute exacerbations of idiopathic pulmonary fibrosis","volume":"10","author":"Ding","year":"2013","journal-title":"Int J Med Sci"},{"key":"2024102403294362000_26.145.170050.86","doi-asserted-by":"publisher","DOI":"10.1016\/j.pupt.2014.04.008"},{"key":"2024102403294362000_26.145.170050.87","doi-asserted-by":"publisher","DOI":"10.1159\/000369828"},{"key":"2024102403294362000_26.145.170050.88","first-page":"5755","article-title":"Efficacy of thrombomodulin for acute exacerbation of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia: a nonrandomized prospective study","volume":"9","author":"Abe","year":"2015","journal-title":"Drug Des Devel Ther"},{"key":"2024102403294362000_26.145.170050.89","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(03)00600-7"},{"key":"2024102403294362000_26.145.170050.90","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2013.06.039"},{"key":"2024102403294362000_26.145.170050.91","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2011.03684.x"},{"key":"2024102403294362000_26.145.170050.92","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2009.09.031"},{"key":"2024102403294362000_26.145.170050.93","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0b013e31824d7079"},{"key":"2024102403294362000_26.145.170050.94","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2010.08.006"},{"key":"2024102403294362000_26.145.170050.95","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2012.12.067"},{"key":"2024102403294362000_26.145.170050.96","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2012.12.022"},{"key":"2024102403294362000_26.145.170050.97","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2009.12.021"},{"key":"2024102403294362000_26.145.170050.98","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.45.6018"},{"key":"2024102403294362000_26.145.170050.99","first-page":"113","article-title":"Polymyxin-B hemoperfusion for acute exacerbation of idiopathic pulmonary fibrosis: serum IL-7 as a prognostic marker","volume":"28","author":"Tachibana","year":"2011","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis"},{"key":"2024102403294362000_26.145.170050.100","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.51.6965"},{"key":"2024102403294362000_26.145.170050.101","doi-asserted-by":"crossref","first-page":"e0127771","DOI":"10.1371\/journal.pone.0127771","article-title":"Autoantibody-targeted treatments for acute exacerbations of idiopathic pulmonary fibrosis","volume":"10","author":"Donahoe","year":"2015","journal-title":"PLoS ONE"},{"key":"2024102403294362000_26.145.170050.102","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1113354"},{"key":"2024102403294362000_26.145.170050.103","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1186\/s12890-015-0146-4","article-title":"Survival in idiopathic pulmonary fibrosis acute exacerbations: the non-steroid approach","volume":"15","author":"Papiris","year":"2015","journal-title":"BMC Pulm Med"}],"updated-by":[{"DOI":"10.1183\/16000617.5050-2017","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2018,4,13]],"date-time":"2018-04-13T00:00:00Z","timestamp":1523577600000}}],"container-title":["European Respiratory Review"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/16000617.0050-2017","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T04:59:49Z","timestamp":1740373189000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/16000617.0050-2017"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,9,27]]},"references-count":103,"journal-issue":{"issue":"145","published-online":{"date-parts":[[2017,9,27]]},"published-print":{"date-parts":[[2017,9,30]]}},"alternative-id":["10.1183\/16000617.0050-2017"],"URL":"https:\/\/doi.org\/10.1183\/16000617.0050-2017","relation":{"correction":[{"id-type":"doi","id":"10.1183\/16000617.5050-2017","asserted-by":"object"}]},"ISSN":["0905-9180","1600-0617"],"issn-type":[{"value":"0905-9180","type":"print"},{"value":"1600-0617","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,9,27]]}}}