RT Journal Article
SR Electronic
T1 Factors associated with FEV1 decline in cystic fibrosis: analysis of the data of the ECFS Patient Registry
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP erj01664-2012
DO 10.1183/09031936.00166412
A1 Eitan Kerem
A1 Laura Viviani
A1 Anna Zolin
A1 Stephanie MacNeill
A1 Elpis Hatziagorou
A1 Helmut Ellemunter
A1 Pavel Drevinek
A1 Vincent Gulmans
A1 Uros Krivec
A1 Hanne Olesen
A1 on behalf of the ECFS Patient Registry Steering Group
YR 2013
UL http://erj.ersjournals.com/content/early/2013/04/17/09031936.00166412.abstract
AB Pulmonary insufficiency is the main cause of death in cystic fibrosis (CF). The European CF Society Patient Registry (ECFSPR) offers a unique database to evaluate potential risk factors of CF lung disease in a heterogeneous population.We analysed forced expiratory volume in one second (FEV1%) data of 14732 patients registered in the ECFSPR in year 2007. Linear and logistic regressions were used to investigate association between FEV1% and age, age at diagnosis, sex, pancreatic status, chronic P. aeruginosa infection, CF-related diabetes (CFRD), BMI, and genotype. Estimates were adjusted for country.BMI, chronic infection by P. aeruginosa, pancreatic status and CFRD showed a statistically significant (all p<0·0001) and clinically relevant effect on FEV1%. Age at diagnosis, sex and genotype had a statistically significant (p<0·01), but not clinically relevant effect. We estimate that patients with lower BMI experience a 6 fold increased odds (95% CI 5·0;7.3) of having severe lung disease (FEV1<40%) compared to patients with normal BMI, and that patients affected by CFRD have a 1·8 fold increased odds (1·6;2·2) compared to patients not affected. Being chronically infected with P. aeruginosa increases the odds of severe lung disease by 2·4 (2·0;2·7) and pancreatic insufficient patients experience a 2·0 fold increased odds (1·6;2·5) of severe lung disease compared to pancreatic sufficient patients.Nutrition, chronic P. aeruginosa infection, pancreatic status and CFRD were found to be potential risk factors of pulmonary function in patients with CF regardless of age. Since three of these factors are preventable or treatable, we emphasize the importance of their early identification through frequent routine tests, the implementation of infection control measures, and a timely initiation of antibiotic therapies. Furthermore, epidemiological and clinical studies comparing CF patients from different populations should control for these factors to avoid confounding.