Alpha-1 Antitrypsin Deficiency — A Missed Opportunity in COPD?
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|Pathogenesis of COPD in MZ heterozygotes. A normal protease/antiprotease balance exists in MM individuals (left panel). Non-smoking MZ individuals have intermediate levels of AAT and increased sputum IL-8 levels and neutrophil counts (middle panel). Reactive oxygen species in cigarette smoke inactivate AAT resulting in a protease/antiprotease imbalance with increased amounts of neutrophil elastase. Polymerisation of Z AAT protein and increased amounts of IL-8 increase neutrophil influx into the MZ lung. An overwhelmed anti-protease defence contributes to the development of COPD (right panel).|