Cardiovascular and musculskeletal co-morbidities in patients with alpha 1 antitrypsin deficiency.
BACKGROUND
Determining the presence and extent of co-morbidities is fundamental in assessing patients with chronic respiratory disease, where increased cardiovascular risk, presence of osteoporosis and low muscle mass have been recognised in several disease states. We hypothesised that the systemic consequences are evident in a further group of subjects with COPD due to Alpha-1 Antitrypsin Deficiency (A1ATD), yet are currently under-recognised.


METHODS
We studied 19 patients with PiZZ A1ATD COPD and 20 age, sex and smoking matched controls, all subjects free from known cardiovascular disease. They underwent spirometry, haemodynamic measurements including aortic pulse wave velocity (aPWV), an independent predictor or cardiovascular risk, dual energy X-ray absorptiometry to determine body composition and bone mineral density.


RESULTS
The aPWV was greater in patients: 9.9(2.1) m/s than controls: 8.5(1.6) m/s, p = 0.03, despite similar mean arterial pressure (MAP). The strongest predictors of aPWV were age, FEV1% predicted and MAP (all p < 0.01). Osteoporosis was present in 8/19 patients (2/20 controls) and was previously unsuspected in 7 patients. The fat free mass and bone mineral density were lower in patients than controls (p < 0.001).


CONCLUSIONS
Patients with A1ATD related COPD have increased aortic stiffness suggesting increased risk of cardiovascular disease and evidence of occult musculoskeletal changes, all likely to contribute hugely to overall morbidity and mortality.
Section of Respiratory Medicine, Wales Heart Research Institute, School of Medicine, Cardiff University, Heath Park, Cardiff, UK.
James M
JM
Duckers
Dennis J
DJ
Shale
Robert A
RA
Stockley
Nichola S
NS
Gale
Bronwen A J
BA
Evans
John R
JR
Cockcroft
Charlotte E
CE
Bolton
D017677:Age Distribution; D002318:Cardiovascular Diseases / Q000401:mortality*; D015897:Comorbidity; D005260:Female; D006801:Humans; D007592:Joint Diseases / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D029424:Pulmonary Disease, Chronic Obstructive / Q000401:mortality*; D018570:Risk Assessment; D012307:Risk Factors; D017678:Sex Distribution; D016019:Survival Analysis; D015996:Survival Rate; D006113:United Kingdom / Q000453:epidemiology; D019896:alpha 1-Antitrypsin Deficiency / Q000401:mortality*