Key current clinical issues in alpha-1 antitrypsin deficiency.

Alpha-1 antitrypsin deficiency is a common but under-recognized condition on which respiratory therapists can have a large impact. A key recent development is the issuance of an international evidence-based standards document regarding diagnosis and management of individuals with alpha-1 antitrypsin deficiency. This report summarizes that standards document, which recommends more widespread testing for alpha-1 antitrypsin deficiency, in order to extend the benefits of diagnosis to individuals found to have alpha-1 antitrypsin deficiency. An important aspect of the standards document is that evidence regarding the clinical efficacy of intravenous alpha-1 antitrypsin augmentation therapy is reviewed. Though no definitive support from a randomized clinical trial is available, the weight of evidence favors the clinical efficacy of alpha-1 antitrypsin augmentation therapy, at least in individuals with moderate degrees of established airflow obstruction. On that basis the standards document recommends intravenous augmentation therapy in specified clinical circumstances.