Increased augmentation index in patients with cystic fibrosis

Abstract

Increased large artery stiffness occurs in a range of inflammatory conditions indicating an ageing of the vasculature and additionally being an independent risk factor for cardiovascular events. We determined large artery parameters in adults with cystic fibrosis (CF).50 clinically stable adult patients with CF (mean¡SD age 28.0¡8.2 yrs) and 26 controls matched for age, sex and body mass index were studied. Central aortic blood pressure, augmentation index (AIx) and aortic pulse wave velocity (PWV) were determined using applanation tonometry. Lung function, diabetic status and C-reactive protein (CRP) were also determined.Mean¡SD AIx was greater in patients than controls, 8.5¡11.1% and -1.8¡13.1%, respectively (p,0.001), while PWV was similar. Although AIx was greatest in the sub-group with CF-related diabetes (CFRD), it was also increased in the non-CFRD sub-group when compared with controls. In patients, AIx was related to log 10 CRP (r50.33) and forced vital capacity (r5 -0.34; both p,0.05), and CRP remained predictive in multiple regression.AIx is increased in adults with CF, in the presence of a normal blood pressure and independent of diabetic status. AIx was related to the systemic inflammatory status. These findings have implications for management and require further exploration so that cardiovascular health can be maintained.

Increased arterial stiffness in children with cystic fibrosis: Table 1–

“…It remains unclear whether these interventions completely normalise the increase in arterial stiffness or the vascular changes remain permanently altered. The results of the present exploratory study, together with the results of other studies [4,10], suggest that aggressive and early anti-inflammatory therapies are indicated in CF patients not only to stabilise lung function but also to avoid extrapulmonary complications and to reduce the accelerated vascular ageing process.…”

“…The increase in arterial stiffness seems to be related to systemic inflammation, as expressed by increased IgG and resulting from colonisation with pathogens and irrespective of blood pressure or diabetes status. Conversely, in adult CF patients, CFRD seems to influence arterial stiffness [4]. This discrepancy between adults and children may indicate that the changes in the arterial wall architecture appear only after decades of hyperglycaemic burden.…”

“…Diseases with a systemic inflammatory state are associated with increased arterial stiffness, an independent risk factor for cardiovascular diseases [2,3]. This premature ageing of the vasculature has been demonstrated in CF adults, but it is not known whether it begins in childhood [4]. We hypothesised that arterial stiffness is increased in CF children compared with healthy controls and that it is related to the degree of systemic inflammation.…”

Premature vascular ageing in cystic fibrosis

“…There is very little clinical or epidemiological data linking increased cardiovascular risk in CF patients, perhaps due to the shortened lifespan in these patients or to protection against the development of cardiovascular disease due to a favourable lipid and blood pressure profile in these patients [11]. In this issue of the European Respiratory Journal, HULL et al [12] suggest that the presence of systemic inflammation in CF patients may lead to vascular changes, specifically an increase in vascular stiffness, which may predict future increased cardiovascular risk that might become apparent with the increase in longevity in these patients.…”

“…In a cross-sectional study, 50 adult patients with CF and a mean¡SD age of 28.0¡8.2 yrs had their arterial stiffness measured by aortic (carotid-femoral) and brachial (carotidradial) pulse wave velocity (PWV) [12]. In addition, the central arterial wave form was analysed to calculate the augmentation index (AIx), which is another measure of arterial stiffness [13].…”