TOPLINE:

In patients with cystic fibrosis (CF), the occurrence of pulmonary exacerbations requiring intensive therapy (PERITs) was associated with marked decline in lung function that was not always reversed by intravenous antibiotic therapy. The magnitude of the decline and the pattern of subsequent recovery were similar regardless of using CF transmembrane conductance regulator modulator therapy.

METHODOLOGY:

• Researchers analyzed children and adults with CF from 11 specialist treatment centers across Australia to characterize treatments and lung function outcomes of PERITs.
• A total of 982 patients were enrolled, contributing 593 episodes of PERITs; PERITs were defined as pulmonary exacerbations characterized by acute or subacute deterioration in lung function or symptoms necessitating intravenous antibiotic therapy.
• Respiratory samples for identification of pathogens were collected from 14 days before up to 48 hours after onset of PERITs; lung function was measured at baseline, day 0, day 7, and day 60 following onset of a PERIT.
• Management and outcomes of PERITs were compared between patients who did vs did not receive CF transmembrane conductance regulator modulators.

TAKEAWAY:

• Overall, the median age at onset of a PERIT was 12 years; 77% of PERITs occurred in children younger than 18 years, and the mean percent-predicted forced expiratory volume in 1 second at baseline across PERITs was 80%.
• The most commonly used primary antibiotic was intravenous piperacillin-tazobactam, and the most frequently used adjunctive agent was intravenous and/or inhaled tobramycin.
• Among patients with spirometry data at baseline and follow-up, 41% had not completely recovered their baseline lung function by day 7, and 44% had not fully recovered by day 60; the findings were similar between groups, irrespective of baseline lung function, Pseudomonas aeruginosa colonization, or the use of modulator therapy.
• Patients’ likelihood of recovering at least 90% of their baseline lung function was similar regardless of age, baseline lung function, P aeruginosa colonization, or the use of modulator therapy.

IN PRACTICE:

“This [study] underscores the need for clinicians to approach the management of PERITs in people receiving CFTR [cystic fibrosis transmembrane conductance regulator] modulators with the same vigilance and therapeutic intensity as they would for those not receiving modulators,” the authors of the study wrote.

SOURCE:

This study was led by Parveen Fathima, Health and Clinical Analytics, Sydney School of Public Health, The University of Sydney, Sydney, Australia. It was published online on October 09, 2025, in European Respiratory Journal.

LIMITATIONS:

Some patients had more than one exacerbation, which may have affected the independence of data. Incomplete spirometry data at all timepoints might have biased the recovery analyses. The study did not adjust for confounding factors and could not establish causal inferences. It included only exacerbations treated with intravenous antibiotics and did not capture clinicians’ rationale for treatment decisions, and thresholds for intravenous therapy may vary across clinical settings. 

DISCLOSURES:

This study received support from the BHP Blue Sky Award, the MTPConnect Project Fund Program, and the Australian Medical Research Future Fund. The authors reported having no conflicts of interest.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.