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Lookup NU author(s): Maryam Almulhem, Professor Christopher WardORCiD, Dr Iram Haq, Dr Malcolm Brodlie
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ. BACKGROUND: Pulmonary exacerbations (PExs) are clinically important in people with cystic fibrosis (CF). Multiple definitions have been used for PEx, and this scoping review aimed to identify the different definitions reported in the literature and to ascertain which signs and symptoms are commonly used to define them. METHODS: A search was performed using Embase, MEDLINE, Cochrane Library, Scopus and CINAHL. All publications reporting clinical trials or prospective observational studies involving definitions of PEx in people with CF published in English from January 1990 to December 2022 were included. Data were then extracted for qualitative thematic analysis. RESULTS: A total of 14 039 records were identified, with 7647 titles and abstracts screened once duplicates were removed, 898 reviewed as full text and 377 meeting the inclusion criteria. Pre-existing definitions were used in 148 publications. In 75% of papers, an objective definition was used, while 25% used a subjective definition, which subcategorised into treatment-based definitions (76%) and those involving clinician judgement (24%). Objective definitions were subcategorised into three groups: those based on a combination of signs and symptoms (50%), those based on a predefined combination of signs and symptoms plus the initiation of acute treatment (47%) and scores involving different clinical features each with a specific weighting (3%). The most common signs and symptoms reported in the definitions were, in order, sputum production, cough, lung function, weight/appetite, dyspnoea, chest X-ray changes, chest sounds, fever, fatigue or lethargy and haemoptysis. CONCLUSION: We have identified substantial variation in the definitions of PEx in people with CF reported in the literature. There is a requirement for the development of internationally agreed-upon, standardised and validated age-specific definitions. Such definitions would allow comparison between studies and effective meta-analysis to be performed and are especially important in the highly effective modulator therapy era in CF care.
Author(s): Almulhem M, Ward C, Haq I, Gray RD, Brodlie M
Publication type: Review
Publication status: Published
Journal: BMJ Open Respiratory Research
Year: 2024
Volume: 11
Issue: 1
Online publication date: 15/08/2024
Acceptance date: 17/07/2024
ISSN (electronic): 2052-4439
Publisher: BMJ Publishing Group
URL: https://doi.org/10.1136/bmjresp-2024-002456
DOI: 10.1136/bmjresp-2024-002456
PubMed id: 39147400
Data Access Statement: All data relevant to the study are included in the article or uploaded as supplementary information.
