Alpha-1 Antitrypsin Deficiency: Treatment Beyond Augmentation Therapy

Author(s): Amit Mahajan, D. Kyle Hogarth

Journal Name: Anti-Inflammatory & Anti-Allergy Agents in Medicinal Chemistry
Formerly Current Medicinal Chemistry - Anti-Inflammatory & Anti-Allergy Agents

Volume 9 , Issue 4 , 2010

Become EABM
Become Reviewer
Call for Editor


Alpha-1-antitrypsin (AAT) deficiency is a debilitating disease characterized by progressive parenchymal lung destruction. Deficiency of AAT results in a protease-antiprotease imbalance with unregulated activity of neutrophil elastase (NE). This imbalance results in accelerated parenchymal lung damage and a subsequent decrease in pulmonary function. Repletion of AAT enzyme with augmentation therapy using human pooled AAT is the current focus of treatment for AAT deficiency. While augmentation is a cornerstone of therapy for individuals with AAT deficiency, practitioners must also maximize standard treatment for obstructive lung disease like chronic obstructive pulmonary disease (COPD) to provide improved quality of life in patients suffering from AAT deficiency. Interventions such as smoking cessation, inhaled medications, pulmonary rehabilitation, supplemental oxygen therapy, vaccinations, avoidance of environmental exposures, and prophylactic antibiotics are essential therapies for the management of AAT deficiency.

Keywords: Alpha-1-antitrypsin, alpha-1-antitrypsin deficiency, alpha-1-antitrypsin deficiency treatment. augmentation therapy, smoking cessation, pulmonary rehabilitation.

Rights & PermissionsPrintExport Cite as

Article Details

Year: 2010
Page: [330 - 335]
Pages: 6
DOI: 10.2174/1871523011009040330
Price: $65

Article Metrics

PDF: 7