Augmentation Therapy for Emphysema due to Alpha-1 Antitrypsin Deficiency: Pro

Prevalence of α1-antitrypsin PiZZ genotypes in patients with COPD in Europe: a systematic review

“…number of individuals with the PiZZ genotype who develop COPD) reaches at most 60% [2,5,7]. This is an important fact to consider, as severe AAT deficiency is the only subtype of COPD with a specific augmentative therapy with infusions of purified plasma-derived AAT [13]; hence the importance of knowing the number of patients with PiZZ at high risk of developing COPD in each country, in order to enhance awareness of this association among healthcare providers and the general public, for planning health policies and financial medical resources and their utilisation by the scientific community, governments and pharmaceutical industry [11,14].…”

Augmentation Therapy Modulates Systemic Inflammation in Individuals with Alpha-1 Antitrypsin Deficiency and Chronic Obstructive Pulmonary Disease

“…13 Study Design and Methods individuals with COPD by maintaining circulating levels of AAT and effective inhibition of neutrophil elastase in the lung. 12 In this study, we examined the effect of AAT augmentation therapy on the systemic inflammation in individuals with COPD due to AATD using plasma levels of CRP as our main outcome.…”

Treatment with inhaled α1-antitrypsin: a square peg in a round hole?

“…In the best-case scenario, an improvement in FEV 1 within the range of less than 100 mL can also be obtained with other drugs such as ICS, which are less expensive and more convenient than inhaled AAT [24]. A possible lack of (or small) effect of AAT on FEV 1 should not mislead us from recognising the crucial effect of augmentation therapy in reducing the rate of destruction of lung parenchyma in emphysema associated with AATD [25][26][27].…”