Diagnosis and Management of Cystic Fibrosis Exacerbations

Tijana Milinic; Oliver J. McElvaney; Christopher H. Goss

doi:10.1055/s-0042-1760250

Seminars in Respiratory and Critical Care Medicine, Table of Contents

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

¹Department of Medicine, University of Washington School of Medicine, Seattle, Washington

,

Oliver J. McElvaney

²Cysic Fibrosis Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington

,

Christopher H. Goss

¹Department of Medicine, University of Washington School of Medicine, Seattle, Washington

²Cysic Fibrosis Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington

³Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington

› Author Affiliations

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

Full Text

References

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