Background: Cystic Fibrosis (CF) is the most common autosomal recessive condition affecting Caucasians. Improved survival paralleled with a high prevalence of risk factors (diabetes and systemic inflammation) suggest that cardiovascular disease may become an important comorbidity in CF, yet currently cardiovascular risk profiling is lacking. We hypothesised that augmentation index (Aix), a marker of arterial stiffness, would be increased in CF.

Methods: We studied 50 (33 male) adults with stable CF, mean (range) age 28.0 (16–46) yrs and 26 age/gender/BMI matched controls. We measured heart rate adjusted Aix, aortic pulse wave velocity (PWV) (SphygmoCor), blood pressure, spirometry (FEV₁ & FVC), glucose tolerance status, serum CRP and lipids.

Results: Height, MAP and PWV were similar however Aix was increased in patients and the difference to controls persisted following adjustment for gender, age, MAP and height. Aix was greatest in diabetic patients (n=13), 13.1 (4.3)%, but the non-diabetic sub-group still had greater Aix: 6.9(12.3)% than controls (ANOVA, p<0.05). CRP was increased in patients (p<0.005). In patients, Aix was inversely related to FEV₁ (r=−0.43) and FVC (r=−0.54), and directly with age (r=0.54), all p<0.01 and log₁₀CRP (r=0.33, p<0.05).

Conclusions: Aix was increased in adults with CF, independent of glycaemic status. This apparent vascular ageing may have important implications for surveillance and cardiovascular risk stratification of these patients.