8.5 INCREASED ARTERIAL STIFFNESS IN PATIENTS WITH ALPHA 1 ANTITRYPSIN DEFICIENCY

Abstract

Rationale: Alpha 1 Antitrypsin (AAT) deficiency is a familial cause of emphysema, due to reduced antiproteolytic activity within lungs. Systemic manifestations however, remain little explored. We have previously demonstrated increased arterial stiffness (AS) in patients with COPD and hypothesised that AAT deficient patients would present similarly but with lesser smoking exposure. Methods: 19 AAT deficient patients, and 20 age, sex and smoking matched controls, all free of known cardiovascular disease were studied at clinical stability. All underwent spirometry (FEV1), large artery haemodynamics including aortic pulse wave velocity (PWV), DEXA (body composition) and blood sampling (IL-6 & lipids). Results: Age, heart rate, lipids and MAP were similar. Aortic PWV was greater in patients, than controls. Significant independent variables of aortic PWV in all subjects were age, FEV1% and MAP, all p<0.001. BMI and Fat free mass index (skeletal muscle mass) were lower in patients than controls, p<0.001. Conclusions: AAT deficient patients have increased AS, as determined by aortic PWV, which supports they are at increased cardiovascular risk. Mean (SD) Control (n=20) Patient (n=19) Men n (%) 13 (65) 12 (63) Age (yrs) 61.1 (9.1) 59.2(12.1) Smoke Pack yrs § 5.5 (0–70) 10.0 (0–60) FEV1 (% predicted) 100.8 (12.5) 42.7 (23.3)** Heart rate (bpm) 68.2 (12.4) 75.7 (12.5) MAP (mmHg) 100.4 (10.2) 101.5 (9.2) Aortic PWV (m/s) 8.5 (1.6) 9.9 (2.1)* AIx (%) 23.7 (8.8) 26.1 (6.5) IL-6 (pg/ml) † 2.18 (1.64) 3.24 (1.62)* *p<0.05; **p<0.001. §median (range). †geometric mean (SD)