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Semin Respir Crit Care Med 2023; 44(02): 225-241
DOI: 10.1055/s-0042-1760250
Review Article

Diagnosis and Management of Cystic Fibrosis Exacerbations

Tijana Milinic
1   Department of Medicine, University of Washington School of Medicine, Seattle, Washington
,
Oliver J. McElvaney
2   Cysic Fibrosis Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington
,
Christopher H. Goss
1   Department of Medicine, University of Washington School of Medicine, Seattle, Washington
2   Cysic Fibrosis Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington
3   Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington
› Author Affiliations Funding C.H.G.'s research time is supported by the Cystic Fibrosis Foundation Therapeutics (grant no.: GOSS13A0) and NIH (grant numbers: P30 DK089507 and UL1TR000423). There was no role of funding sources in the writing of this manuscript or the decision to submit for publication.
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Abstract

With the improving survival of cystic fibrosis (CF) patients and the advent of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) therapy, the clinical spectrum of this complex multisystem disease continues to evolve. One of the most important clinical events for patients with CF in the course of this disease is acute pulmonary exacerbation (PEx). Clinical and microbial epidemiology studies of CF PEx continue to provide important insight into the disease course, prognosis, and complications. This work has now led to several large-scale clinical trials designed to clarify the treatment paradigm for CF PEx. The primary goal of this review is to provide a summary and update of the pathophysiology, clinical and microbial epidemiology, outcome and treatment of CF PEx, biomarkers for exacerbation, and the impact of highly effective modulator therapy on these events moving forward.

Keywords

FEV1 - respiratory symptoms - pulmonary exacerbations - cystic fibrosis - treatment duration


Publication History

Article published online:
06 February 2023

© 2023. Thieme. All rights reserved.

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