Evaluation of Rescue Oral Glucocorticoid Therapy during Inpatient Cystic
Acute pulmonary exacerbation (APE) in CF is characterized by increased
pulmonary symptoms attributed to an increase in inflammation.
Antimicrobials, airway clearance and nutritional support remain the
mainstay of therapy. However, when patients fail to improve,
corticosteroids have been reported as an adjunct therapy. We
retrospectively examined the use of rescue steroids in a children’s
hospital during CF APE following at least one week of inpatient therapy
without expected improvement from 2013 - 2017. 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 admissions not given steroids
change in FEV1pp=20.1, ±24.6; p-value<0.001. Failure to
improve as expected was documented 98% of the time as the rationale for
steroid use. At discharge, there was no difference in mean FEV1pp
(p=0.76). Propensity matching was also evaluated and revealed no
difference in admission, midpoint, or discharge FEV1pp between groups.
Equally, no difference in FEV1pp at follow-up visit or in time until
next APE was detected between groups. Moreover, delay in steroid therapy
by waiting until the end of the second week increased length of stay.
Secondary analysis for associations including gender, genotype, fungal
colonization, or inhaled antimicrobials were non-significant. Our data
suggest rescue use of corticosteroids during APE does not predictably
impact important outcome measures in CF APE.