title: Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm.
abstract: The goal of this review is to summarise the clinical features, management, and prognosis of acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF). AE-IPF has previously been defined based on clinical and radiological features that include the subacute onset of dyspnoea, bilateral ground glass changes on chest high-resolution computed tomography, and the absence of an identifiable aetiology. The annual incidence of AE-IPF is typically reported at 5-15%, 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 ~50%. 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.
authors.affiliation: Dept of Medicine, University of British Columbia, Vancouver, BC, Canada.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Ryerson
authors.affiliation: Service de Pneumologie, Centre national de référence des maladies pulmonaires rares, Hôpital Louis Pradel, Université Claude Bernard Lyon 1, Lyon, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.affiliation: Dept of Medicine, National Jewish Health, Denver, CO, USA.
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.affiliation: Dept of Medicine, University of California San Francisco, San Francisco, CA, USA hal.collard@ucsf.edu.
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
mesh_terms: D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D001706:Biopsy; D018450:Disease Progression; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000401:mortality*; D015994:Incidence; D011379:Prognosis; D011650:Pulmonary Alveoli / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed
chemical_list: D000305:Adrenal Cortex Hormones