An acute pulmonary exacerbation (APE) in cystic fibrosis (CF) is characterized by increased pulmonary symptoms attributed to bacterial colonization, neutrophil recruitment, and inflammation. Antimicrobials, airway clearance, and nutrition are the mainstay of therapy. However, when patients fail to improve, corticosteroids have been added to therapy. We retrospectively examined the use of rescue steroids in a children's hospital from 2013 to 2017 during CF APE treatment following at least 1 week of inpatient therapy without expected clinical improvement. In total, 106 encounters of 53 unique patients, aged 6–20 years, who had FEV1 percent predicted (FEV1pp) data at baseline, admission, midpoint, and discharge, and had admission duration of at least 12 days were studied. Encounters treated with steroids had less improvement at midpoint percent change from admission in FEV1pp (4.9 ± 11.3) than nonsteroid group change in FEV1pp (20.1 ± 24.6; p <.001). Failure to improve as expected was the rationale for steroid use. At discharge, there was no difference in mean FEV1pp (p =.76). Delays in steroid therapy by waiting until the end of the second week increased the total length of stay (LOS). Propensity matching, comparing outcomes in patients without midpoint improvement in FEV1pp, was also evaluated. There was no difference in admission or discharge FEV1pp between groups. Equally, no difference in FEV1pp at follow-up visit or in time until the next APE was detected. Secondary analysis for associations including gender, genotype, fungal colonization, or inhaled antimicrobials was nonsignificant. These data suggest rescue use of corticosteroids during APE does not predictably impact important outcome measures during CF APE treatment.
- cystic fibrosis
- pulmonary exacerbation
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Pulmonary and Respiratory Medicine