Alpha-1 antitrypsin deficiency as a common treatable mechanism in chronic respiratory disorders and for conditions different from pulmonary emphysema? A commentary on the new European Respiratory Society statement

作者：Andrea Gramegna, Stefano Aliberti, Marco Confalonieri, Angelo Guido Corsico, Luca Richeldi, Carlo Vancheri, Francesco Blasi · 发表于：Multidisciplinary Respiratory Medicine · 年份：2018 · DOI：10.1186/s40248-018-0153-4 · 被引用次数：20 · 研究领域：Protease and Inhibitor Mechanisms、Pulmonary Hypertension Research and Treatments、Neutrophil, Myeloperoxidase and Oxidative Mechanisms

BACKGROUND: The European Respiratory Society recently published an important statement reviewing available evidence on diagnosis and treatment of lung disease associated to alpha-1 antitrypsin deficiency (AATD). Several issues on this topic still remain unresolved and subject of interpretation according to different standard procedures and healthcare systems worldwide. The purpose of this commentary is to offer a critical contribution to most of these controversial issues in light of an Italian perspective for the management of this disease. MAIN BODY: The clinical spectrum of AATD lung disease might include different manifestations and the traditional paradigm of a younger emphysematous patient has been revealing insufficient. Targeting with appropriate testing only COPD patients might be considered a limited approach leading to underestimation of the real prevalence of the disease. Several reports have suggested the association between AATD and other chronic respiratory conditions, as asthma and bronchiectasis. A deeper evaluation of clinical, radiological, microbiological and functional variables is, therefore, needed in order to investigate different phenotypes in AATD patients. In addition, a new line of translational research in AATD might focus on the development of personalized therapeutic regimens taking into account the patient clinical profile and needs. CONCLUSIONS: Over the past years, AATD has been interpreted as a common mechanism of inflammatory disequilibrium...