Cardiovascular disease in Alpha 1 antitrypsin deficiency: an observational study assessing the role of neutrophil proteinase activity and the suitability of validated screening tools.
Elizabeth SapeyL E CrowleyRoss G EdgarD GriffithsS SamantaH CrisfordCharlotte Emma BoltonJohn R HurstRobert A StockleyPublished in: Orphanet journal of rare diseases (2024)
aPWV measured CVD risk is elevated in AATD. This risk is not captured by QRISK2®. There is a relationship between aPWV, lung disease and proteinase-3 activity. Proteinase-driven breakdown of elastin fibres in large arteries and lungs is a putative mechanism and forms a potential therapeutic target for CVD in AATD.