Ann Am Thorac Soc. 2022 Aug 31. doi: 10.1513/AnnalsATS.202203-201OC. Online ahead of print.
ABSTRACT
RATIONALE: Pulmonary exacerbation (PEx) events contribute to lung function decline in people with cystic fibrosis (CF). CF Foundation PEx guidelines note a short course of systemic corticosteroids may offer benefit without contributing to long-term adverse effects. However, insufficient evidence exists to recommend systemic corticosteroids for PEx treatment.
OBJECTIVES: To determine if systemic corticosteroids for the treatment of in-hospital pediatric PEx is associated with improved clinical outcomes compared to treatment without systemic corticosteroids.
METHODS: Retrospective cohort study using the CF Foundation Patient Registry-Pediatric Health Information System linked database. People with CF were included if hospitalized for a PEx between 2006-2018 and were 6-21 years of age. Time to next PEx was assessed by Cox proportional hazards regression. Lung function outcomes were assessed by linear mixed effect modeling and generalized estimating equations. To address confounding by indication, inverse probability treatment weighing was used.
RESULTS: 3,471 people with CF contributed 9,787 PEx for analysis. Systemic corticosteroids were used in 15% of all PEx. In our primary analysis, systemic corticosteroids were not associated with better pre- to post-PEx percent predicted forced expiratory volume in 1 second responses (mean difference, -0.36, 95% CI: -1.14, 0.42; p=0.4) or a higher odds of returning to lung function baseline (odds ratio (OR), 0.97, 95% CI: 0.84-1.12; p=0.7), but were associated with a reduced chance of future PEx requiring intravenous antibiotics (hazard ratio (HR), 0.91 (95% CI: 0.85-0.96; p=0.002). When restricting the analysis to one PEx per person, lung function outcomes remained no different among PEx treated with or without systemic corticosteroids, but in contrast to our primary analysis, the use of systemic corticosteroids was no longer associated with a reduced chance of having a future PEx requiring intravenous antibiotics (HR 0.96 (95% CI: 0.86, 1.07; p=0.42).
CONCLUSIONS: Systemic corticosteroid treatment for in-hospital pediatric PEx was not associated with improved lung function outcomes. Prospective trials are needed to better evaluate the risks and benefits of systemic corticosteroid use for PEx treatment in children with CF.
PMID:36044723 | DOI:10.1513/AnnalsATS.202203-201OC
