Acute exacerbations of idiopathic pulmonary fibrosis: Does clinical stratification or steroid treatment matter?

• Published in: Chronic respiratory disease Vol. 16; pp. 1479973119869334 - 1479973119869341
• Main Authors: Cuerpo, Sandra, Moisés, Jorge, Hernández-González, Fernanda, Benegas, Mariana, Ramirez, Jose, Sánchez, Marcelo, Agustí, Àlvar, Sellares, Jacobo
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.01.2019; Sage Publications Ltd
• Subjects: Acute Disease; Adrenal Cortex Hormones - administration & dosage; Adrenal Cortex Hormones - therapeutic use; Adrenocortical hormones; Aged; Aged, 80 and over; Corticosteroides; Disease Progression; Female; Fibrosi pulmonar; Hospital Mortality; Humans; Idiopathic Pulmonary Fibrosis - classification; Idiopathic Pulmonary Fibrosis - drug therapy; Idiopathic Pulmonary Fibrosis - etiology; Idiopathic Pulmonary Fibrosis - mortality; Male; Middle Aged; Mortality; Original Paper; Pulmonary fibrosis; Retrospective Studies; Steroids; Tomography, X-Ray Computed - methods; acute exacerbation; usual interstitial pneumonia; corticosteroid therapy; Idiopathic pulmonary fibrosis
• ISSN: 1479-9731; 1479-9723; 1479-9731
• DOI: 10.1177/1479973119869334

Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is defined as a sudden acceleration of the disease with the appearance of pulmonary infiltrates superimposed on the characteristic pattern of IPF that leads to a significant decline in lung function. It has high in-hospital mortality rates, despite medical treatment with systematic steroids. We sought to investigate whether there were in-hospital mortality differences according to clinical stratification (AE, suspected AE, or AE of known cause) and/or treatment with systemic steroids. We reviewed the clinical characteristics and outcomes of patients with IPF admitted to our hospital during the years 2003–2014 due to a worsening of their clinical status. We identified 50 IPF patients, 9 with AE (18%), 12 with suspected exacerbation (24%), and 29 with AE of known cause (58%), mostly respiratory infections. In-hospital mortality was similar in the three groups (33% vs. 17% vs. 34%, respectively). Likewise, we did not find differences between them with respect to the use of systemic steroids (length of treatment duration or total dose). Nevertheless, there was an independent association between in-hospital mortality and high average daily steroid dose. We did not observe significant differences in prognosis or use of systemic steroids according to current diagnostic stratification groups in patients hospitalized because of an exacerbation of IPF.