Efficacy of augmentation therapy for emphysema associated with α1-antitrypsin deficiency: enough is enough
B Balbi, I Ferrarotti, M Miravitlles - European Respiratory …, 2016 - Eur Respiratory Soc
B Balbi, I Ferrarotti, M Miravitlles
European Respiratory Journal, 2016•Eur Respiratory Socα1-Antitrypsin deficiency (AATD) is an autosomal co-dominant genetic condition which is
widely prevalent in populations of European descent [1]. The pathogenesis of AATD-related
lung manifestations is related to a protease (namely neutrophil elastase)–antiprotease (ie α1-
antitrypsin, AAT) imbalance occurring in the respiratory system. In subjects with AATD this
imbalance is usually caused by an enhanced burden of neutrophils, hence of neutrophil
elastase, in the lung tissue, due to smoking and/or recurrent respiratory infections, and a …
widely prevalent in populations of European descent [1]. The pathogenesis of AATD-related
lung manifestations is related to a protease (namely neutrophil elastase)–antiprotease (ie α1-
antitrypsin, AAT) imbalance occurring in the respiratory system. In subjects with AATD this
imbalance is usually caused by an enhanced burden of neutrophils, hence of neutrophil
elastase, in the lung tissue, due to smoking and/or recurrent respiratory infections, and a …
α1-Antitrypsin deficiency (AATD) is an autosomal co-dominant genetic condition which is widely prevalent in populations of European descent [1]. The pathogenesis of AATD-related lung manifestations is related to a protease (namely neutrophil elastase)–antiprotease (i.e. α1-antitrypsin, AAT) imbalance occurring in the respiratory system. In subjects with AATD this imbalance is usually caused by an enhanced burden of neutrophils, hence of neutrophil elastase, in the lung tissue, due to smoking and/or recurrent respiratory infections, and a variety of lung disorders may develop, predominantly lower lobe panacinar emphysema [2].
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