Elevated Rates and Earlier Onset of Nonpulmonary Comorbidities in Adults with Cystic Fibrosis: A Population-based
Rigya Arya1, Isobel Sharpe2, Stephanie Y Cheng3
1Division of Respirology and.
Annals of the American Thoracic Society
|July 28, 2025
Summary
People with cystic fibrosis (pwCF) experience higher rates of cardiovascular disease, kidney disease, kidney stones, and cancer, often at a younger age compared to the general population. These non-pulmonary comorbidities require integration into CF care models.
Area of Science:
- Medical research
- Epidemiology
- Public health
Background:
- People with cystic fibrosis (pwCF) are living longer, leading to an increase in non-pulmonary comorbidities.
- Understanding the incidence and comparison of these comorbidities with the general population is crucial for evolving care models.
Purpose of the Study:
- To estimate the incidence of emerging non-pulmonary comorbidities in adults with cystic fibrosis (CF).
- To compare the rates of these comorbidities in pwCF versus the general population.
Main Methods:
- Population-based cohort study utilizing Canadian CF Registry and health administrative data from Ontario.
- Identification of cardiovascular disease (CVD), kidney stones, chronic kidney disease (CKD), and cancer using diagnostic codes and registries.
- Poisson regression analysis to determine incidence rates per 1,000 person-years.
Main Results:
- pwCF without lung transplant showed significantly higher age- and sex-adjusted rates for CVD (RR 2.9), CKD (RR 2.1), kidney stones (RR 2.9), and cancer (RR 1.9) compared to the non-CF population.
- These conditions occurred at least 20 years earlier in the CF cohort.
- Post-lung transplant, rates of CVD, kidney stones, and cancer were similar to the non-CF population, but events still occurred earlier.
Conclusions:
- Non-pulmonary comorbidities are prevalent and occur at a younger age in pwCF.
- The findings underscore the necessity of integrating the management of these conditions into comprehensive CF care strategies.
- Early detection and management of non-pulmonary complications are vital for improving long-term outcomes in pwCF.
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