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The annual incidence of AE-IPF is typically reported at 5\u201315%, but is less common in mild disease. Features of diffuse alveolar damage are present when a biopsy is performed. Idiopathic pulmonary fibrosis (IPF) patients with acute respiratory worsening are often initially treated with high dose corticosteroids and antimicrobials; however, there are no clear data to support these therapies, and the short-term mortality of AE-IPF is \u223c50%. Recent studies have shown that the features and prognosis of AE-IPF are similar to other causes of acute respiratory worsening, including infection, aspiration, air pollution and mechanical injury to the alveolar epithelium. Based on this emerging evidence, we propose a novel approach to the classification of acute respiratory worsening events in patients with IPF that focuses on clinical and radiological findings consistent with an underlying pathobiology of diffuse alveolar damage.<\/jats:p>","DOI":"10.1183\/13993003.00419-2015","type":"journal-article","created":{"date-parts":[[2015,8,6]],"date-time":"2015-08-06T19:23:44Z","timestamp":1438889024000},"page":"512-520","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":182,"title":["Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm"],"prefix":"10.1183","volume":"46","author":[{"given":"Christopher J.","family":"Ryerson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin 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