Pulmonary exacerbations and acute declines in lung function in patients with cystic fibrosis

Background: Patients with cystic fibrosis (CF) who experience acute declines in percent predicted FEV1 (ppFEV(1) decreased >= 10% relative to baseline) are often not treated with antibiotics for pulmonary exacerbations (PEx), whereas other patients are treated even when they have not experienced a decline in lung function. Methods: We analyzed 2 patient cohorts using 3 years of Epidemiologic Study of CF data. Cohort 1 (12,837 patients) experienced a >= 10% acute decline in ppFEV(1) (n = 22,898) and Cohort 2 (10,416 patients) had a clinician-diagnosed PEx (n = 20,731). Results: 70.7% of >= 10% decline events were treated with antibiotics; with intravenous antibiotics used 67.1% of the time. 32.0% of clinician-diagnosed PEx declined <10%; with intravenous antibiotics used 36.9% of the time. Conclusions: A clinician's decision to diagnose a PEx and treat with antibiotics often is not defined by measured lung function: a .10% FEV1 decline is not considered an absolute indication of a PEx and the lack of a decline does not contraindicate a PEx. Clinicians appear to use the history of prior PEx plus other variables as factors for diagnosing PEx. (C) 2018 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.