Urinary stone disease prevalence and associations in cystic fibrosis

Cystic fibrosis (CF) may predispose patients to urinary stone disease (USD), but reported prevalence of USD in patients with CF in previous small studies is variable. To date, analysis of risk factors for USD within the CF population has been limited. We studied 29,396 patients in the Cystic Fibrosis Foundation Patient Registry to calculate age and sex-stratified prevalence of USD. For adult patients, we examined age and multivariable-adjusted cross-sectional associations between demographic and clinical factors, CFTR mutation class, and prevalent USD. Prevalence of USD was 0.4% (95% CI 0.3–0.5%) under age 18 years, 3.1% (2.7–3.6%) at 18–24 years, 6.4% (5.8–7.1%) at 25–34 years, 7.5% (6.5–8.5%) at 35–44 years, and 6.7% (5.8–7.8%) at 45 years and older. Prevalence for women was higher than men at younger (< 45 years) but not older ages (P value for interaction < 0.0005). Multivariable odds of prevalent USD were significantly increased for severe CFTR mutations, OR 1.53 (1.14–2.06), diabetes, OR 1.24 (1.03–1.50), hypertension, OR 1.58 (1.29–1.93), and chronic macrolide therapy, OR 1.27 (1.07–1.52). BMI was not associated with USD. USD prevalence in CF is similar to that in the general population. With the exception of BMI, known risk factors for USD in the general population also appear to be important for patients with CF. We identified several novel associations in CF patients, including greater prevalence of USD in individuals with severe CFTR mutations and among young women.