Incidence and risk factors of cancer in individuals with cystic fibrosis in the UK; a case-control study.
File(s)
Accepted version
Author(s)
Type
Journal Article
Abstract
To assess cancer incidence in the UK cystic fibrosis (CF) population and determine the associated risk factors, we undertook a nested case-control study of patients with CF, registered with the UK CF Registry. Each case with a first reported cancer between 1999 and 2017 was matched with up to 4 controls: by age (±2-years) and year of cancer diagnosis. Conditional logistic regressions were adjusted for sex, lung function (FEV1%), CF related diabetes (CFRD), F508del status, transplant status, DIOS, gastro-oesophageal reflux disease, meconium ileus, Pseudomonas aeruginosa infection, pancreatic insufficiency, proton pump inhibitor (PPI) use, IV antibiotic days and BMI. Results: From 12,886 registered patients, 146 (1.1%) cases of malignancy were identified with 14.3% of cases occurring post solid organ transplant. Site of primary cancer was available for 98 patients: 22% were gastro-intestinal in origin (77% lower, 23% upper GI), 13% skin, 13% breast and 11% lymphomas/leukaemia. In univariable analysis, transplantation increased the odds of reporting any cancer by 2.46 times (95%CI: 1.3-4.6). CFRD also increased the odds of reporting any cancer (OR 2.35; CI: 1.37-4.0) and PPI use (OR 2.0; CI 1.28-3.19). In the multivariable models significant associations with CFRD and transplant remained, while PA infection, PPI use and being overweight showed increased, but statistically insignificant risks. The incidence of GI cancer was strongly associated with CFRD (OR=4.04; 1.47-11.1). Conclusions: We observed a high incidence of lower GI cancers in our cohort which was significantly affected by the presence of CFRD. Screening for gastrointestinal cancers could benefit patients at higher risk.
Date Issued
2022-03-01
Date Acceptance
2021-07-05
Citation
Journal of Cystic Fibrosis, 2022, 21 (2), pp.302-308
ISSN
1569-1993
Publisher
Elsevier
Start Page
302
End Page
308
Journal / Book Title
Journal of Cystic Fibrosis
Volume
21
Issue
2
Copyright Statement
© 2021 Elsevier Ltd. All rights reserved. This manuscript is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International Licence http://creativecommons.org/licenses/by-nc-nd/4.0/
Sponsor
Cystic Fibrosis Trust
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/34348871
PII: S1569-1993(21)01302-3
Grant Number
SRC 004
Subjects
Breast
Cancer
Cystic fibrosis
Gastro-intestinal
Registry
Risk
Skin
Publication Status
Published
Coverage Spatial
Netherlands
Date Publish Online
2021-08-01