Factors associated with FEV1decline in cystic fibrosis: analysis of the ECFS Patient Registry
Open accessAbstract
Pulmonary insufficiency is the main cause of death in cystic fibrosis (CF). We analysed forced expiratory volume in 1 s (FEV 1) data of 14 732 patients registered in the European Cystic Fibrosis Society Patient Registry (ECFSPR) database in 2007. We used linear and logistic regressions to investigate associations between FEV 1% predicted and clinical outcomes. Body mass index (BMI), chronic infection by Pseudomonas aeruginosa, pancreatic status and CF-related diabetes (CFRD) showed a statistically significant (all p<0.0001) and clinically relevant effect on FEV 1% pred after adjusting for age. Patients with a lower BMI experience a six-fold increased odds ratio (95% CI 5.0–7.3) of having severe lung disease (FEV 1<40% pred) compared to patients with normal BMI. Being chronically infected with P. aeruginosa increases the odds ratio of severe lung disease by 2.4 (95% CI 2.0–2.7), and patients with pancreatic insufficiency experience a 2.0-fold increased odds ratio (95% CI 1.6–2.5) of severe lung disease compared to pancreatic sufficient patients. Patients with CFRD have a 1.8-fold increased odds ratio (95% CI 1.6–2.2) compared to patients not affected. These potential risk factors for pulmonary disease in patients with CF are to some degree preventable or treatable. We emphasise the importance of their early identification through frequent routine tests, the implementation of infection control measures, and a timely initiation of relevant therapies.
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Independent of FEV1, frequent or severe exacerbations, massive hemoptysis requiring bronchial artery embolization, pneumothorax, malnutrition, low six-minute walk distance, and younger age at advanced disease are associated with increased risk of progression or death in cystic fibrosis.
“Independent of FEV1, the following factors have been associated with increasing risk of disease progression or death: frequent or severe exacerbations, massive hemoptysis requiring bronchial artery embolization, pneumothorax, malnutrition, low six-minute walk distance, and younger age upon development of advanced disease.(247, 252–260)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [258]
The Study population section (page not supplied) associates poor BMI with sixfold higher odds of severe lung disease, defined by FEV1 below 40% predicted. The paper does not establish risk of progression or death independent of FEV1, and it does not assess the other listed factors, including exacerbations, hemoptysis, pneumothorax, six-minute walk distance, or younger age at advanced disease. (LLM, conf=0.87)
Experimental — accuracy not yet measuredInfection or colonization with multidrug-resistant organisms including Burkholderia cepacia complex, Pseudomonas aeruginosa, and nontuberculous mycobacteria, especially M. abscessus, increases lung function decline or death without transplant in advanced cystic fibrosis.
“Infection or colonization with multi-drug resistant organisms including Burkholderia cepacia complex, Pseudomonas aeruginosa, and nontuberculous mycobacteria (M. abscessus in particular) have been shown to increase the rate of lung function decline or death without transplant in advanced CF.(245, 247, 254, 258, 262)”
From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [258]
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References 27 · Cited by 296
- Authors
- Kerem, E., Viviani, L., Zolin, A., MacNeill, S., Hatziagorou, E., Ellemunter, H., Drevinek, P., Gulmans, V., Krivec, U., & Olesen, H.
- Affiliations
- Hadassah Medical Center (IL)
- University of Milan (IT)
- Imperial College London (GB)
- Aristotle University of Thessaloniki (GR)
- Innsbruck Medical University (AT)
- Nederlandse Cystic Fibrosis Stichting (NL)
- Aarhus University Hospital (DK)
- ORCID
- Venue
- European Respiratory Journal
- Publisher
- European Respiratory Society (ERS)
- Published
- 2013-04-18
- Volume
- 43
- Issue
- 1
- Pages
- 125-133
- ISSN
- 0903-1936 (ISSN-L)
- 1399-3003
- OpenAlex
- W2170978652
- Cited by
- 296
- References
- 27
- Citations by year
- 2026: 8 · 2025: 16 · 2024: 15 · 2023: 26 · 2022: 20 · 2021: 42 · 2020: 26 · 2019: 24 · 2018: 19 · 2017: 18 · 2016: 27 · 2015: 31 · 2014: 19 · 2013: 3
- FWCI
- 21.70
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- European Respiratory Journal (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Chiesi Farmaceutici
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Chronic Obstructive Pulmonary Disease (COPD) Research
- Health Sciences › Medicine › Surgery › Transplantation: Methods and Outcomes
- Keywords
- cystic fibrosis, FEV1 decline, pulmonary insufficiency, body mass index, Pseudomonas aeruginosa infection, pancreatic insufficiency, CF-related diabetes, severe lung disease
- MeSH terms
- Adolescent, Adult, Aged, Child, Cystic Fibrosis, Diabetes Mellitus, Female, Forced Expiratory Volume, Humans, Male, Middle Aged, Exocrine Pancreatic Insufficiency, Pseudomonas aeruginosa, Pseudomonas Infections, Registries, Respiratory Insufficiency, Risk Factors, Severity of Illness Index, Body Mass Index, Linear Models, Logistic Models, Odds Ratio, Disease Progression, Young Adult
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