Kidney Failure in Persons With Cystic Fibrosis

Chronic kidney disease (CKD) occurs with increased frequency in people with cystic fibrosis (PwCF). CKD may progress to kidney failure. We sought to describe the prevalence, characteristics, and outcomes of kidney failure in PwCF.

Retrospective cohort study using data supplied by the US Renal Data System (USRDS), a virtually complete record of all US persons with kidney failure.

All unique deidentified persons listed in the USRDS patient and hospitalization Standard Analytic Files from 2014 through 2018.

Estimated prevalence of kidney failure in PwCF and non-CF cohorts. Comparisons between PwCF and people without CF concerning demographic characteristics, primary cause of kidney failure, diabetes, organ transplant status, mortality, and survival since the onset of kidney failure.

Linear and logistic regression, propensity score-matched Kaplan-Meier survival curves, and log-rank tests.

We estimated the prevalence of kidney failure requiring dialysis or transplantation to be 22.6-29.3 times greater in PwCF than in the general US population. Diabetes was the primary cause of kidney failure in 24.3% of PwCF and 42.3% of people without CF (odds ratio, 0.56; 95% CI, 0.44-0.71; P < 0.001). Complications of lung transplantation were the primary cause in 16.9% of PwCF and 0.03% of people without CF (odds ratio, 682; 95% CI, 501-929; P < 0.001). Overall median survival with kidney failure was 12.6 years for PwCF and 11.8 years for people without CF (P = 0.96).

USRDS data are limited to Medicare claims.

PwCF had an increased prevalence of kidney failure relative to the non-CF US population. The profile of primary causes of kidney failure in PwCF differed from that in the non-CF population. Diabetes was half as frequent, and transplants of lungs and other organs were markedly more frequent primary causes in PwCF. Overall median survival of patients with kidney failure was not significantly different between PwCF and matched people without CF.