TOPLINE:

Patients with cystic fibrosis (CF) who did not receive a lung transplant developed cardiovascular disease, kidney problems, and cancer at higher rates than those without CF. Those with CF who did receive a transplant experienced similar rate of these complications compared with transplant recipients without CF, but they occurred about three decades earlier in those with CF.

METHODOLOGY:

• Researchers conducted a population-based cohort study to assess the rates of non-pulmonary comorbidities in patients with CF compared with adults without CF using the Canadian health administrative databases from April 2002 to March 2022.
• They included patients with CF who received (n = 208; median age, 29 years) or did not receive (n = 1435; median age, 19 years) a transplant vs adults without CF who received (n = 1265; median age, 61 years) or did not receive (n = approximately 16.4 million; median age, 34 years) a transplant.
• Outcomes included diagnostic and procedural codes to identify the incidence and relative risk (RR) of cardiovascular disease, chronic kidney disease, symptomatic kidney stones, and cancer.

TAKEAWAY:

• In patients with CF who did not receive a lung transplant, the age- and sex-adjusted rates per 1000 person years were 24.5 (95% CI, 21.5-28.0) for cardiovascular disease, 3.7 (95% CI, 2.7-5.2) for chronic kidney disease, 7.4 (95% CI, 6.1-9.0) for kidney stones, and 5.8 (95% CI, 4.5-7.6) for cancer.
• Patients with CF who did not receive a transplant had significantly higher RRs for cardiovascular disease (RR, 2.94; 95% CI, 2.57-3.35), chronic kidney disease (RR, 2.08; 95% CI, 1.48-2.93), kidney stones (RR, 2.93; 95% CI, 2.41-3.57), and cancer (RR, 1.92; 95% CI, 1.48-2.49) than those without CF.
• Among transplant recipients, rates of cardiovascular disease, kidney stones, and cancer were similar between patients with CF and adults without CF, although these complications occurred about three decades earlier in those with CF.

IN PRACTICE:

“This study has clinical implications highlighting the need for CF care models to identify and address these emerging non-pulmonary comorbidities early to improve care for pwCF [people with CF]. It will continue to be important to monitor rates of these complications in the future, particularly in the context of increased availability of CFTR [CF transmembrane conductance regulator] modulators,” the authors wrote.

SOURCE:

This study was led by Rigya Arya, Division of Respirology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada. It was published online on July 28, 2025, in Annals of the American Thoracic Society.

LIMITATIONS:

Outcomes depended on the accuracy of documented diagnostic and procedural International Classification of Diseases codes. The results were left truncated as outcomes prior to 2002 were not captured. Additionally, although diabetes may have contributed to the development of cardiovascular disease and chronic kidney disease, the study design precluded conclusions about direct causality.

DISCLOSURES:

This study received grant funding from the Canadian Institute of Health Research and Cystic Fibrosis Canada. Additional support was provided by ICES, which receives annual funding from the Ontario Ministry of Health and the Ministry of Long-Term Care. Few authors reported receiving grants from certain pharmaceutical companies and organizations.

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.