Treatment of pulmonary exacerbations in cystic fibrosis

Christabella Ng, Tejaswi Nadig, Alan R. Smyth, Patrick Flume*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

8 Citations (Scopus)

Abstract

Purpose of review: This review will discuss the challenges of defining a pulmonary exacerbations in cystic fibrosis and the key pathogens, which contribute. It will discuss the treatment options currently available and the importance of preventing pulmonary exacerbations.

Recent findings: The basis for treatment of pulmonary exacerbations remains unchanged over the past 15 years and whilst there have been trials exploring alternative antibiotics, there has been little change. However, there are ongoing studies that are expected to establish a platform for identifying best practices. Chronic cystic fibrosis therapies have been shown to reduce pulmonary exacerbations. In the era of new CFTR (cystic fibrosis transmembrane conductance regulator) modulator therapies, the number of pulmonary exacerbations are expected to be even fewer. However, it is unclear whether the other chronic therapies can be discontinued without losing their benefits in reducing exacerbations.

Summary: Although there is no universal definition of a pulmonary exacerbation in cystic fibrosis, proposed definitions have many similarities. We have outlined the current recommendations for treatment of pulmonary exacerbations, including the duration and location of treatments. We have also summarized the key therapies used for prevention of pulmonary exacerbations in cystic fibrosis.

Original languageEnglish
Pages (from-to)679-684
Number of pages6
JournalCurrent Opinion in Pulmonary Medicine
Volume26
Issue number6
DOIs
Publication statusPublished - 01 Nov 2020
Externally publishedYes

Bibliographical note

Funding Information:
P.F. was supported, in part, by the National Center for Advancing Translational Sciences of the National Institutes of Health under Grant Number UL1 TR001450.

Funding Information:
C.N. has no conflicts to report. T.N. has no conflicts to report. A.R.S. has no conflicts to report. P.F. has received grant support from Abbvie, Corbus Pharmaceuticals, Cystic Fibrosis Foundation Therapeutics, Insmed, National Institutes of Health, Novartis, Novoteris, Proteostasis Therapeutics, Savara, Sound Pharmaceuticals, Inc, Vertex Pharmaceuticals, Inc. P.F. has received honoraria for consultation from Arrevus, Chiesi, Corbus Pharmaceuticals, Eloxx Pharmaceuticals, Hill-Rom, Insmed, Ionis Pharmaceuticals, Janssen Research & Development, McKesson, Merck, Novartis, Polyphor, Proteostasis Therapeutics, Santhera, Savara, Vertex Pharmaceuticals, Inc.

Publisher Copyright:
Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.

Keywords

  • cystic fibrosis
  • pulmonary exacerbations
  • treatment

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

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