The information contained in this page is intended for U.S. healthcare professionals only.
Although there is no cure for AAT deficiency, patients with clinically evident emphysema are candidates for AAT augmentation therapy.1 AAT augmentation therapy with alpha1-proteinase inhibitor involves replacement of the missing protein through infusions of purified human AAT.
Augmentation therapy is recommended by ATS/ERS for "individuals with established airflow obstruction from AAT deficiency."1 Augmentation therapy has been shown to maintain serum levels of AAT above the ATS/ERS-recommended protective threshold of 11 µM.2
Management of obstructive lung disease in individuals with AAT deficiency is similar to management of other obstructive lung diseases, including use of:
- Inhaled bronchodilators
- Supplemental oxygen, when needed
- Antibiotic therapy for respiratory infections
- Brief courses of systemic corticosteroids during acute exacerbations
- Pulmonary rehabilitation for individuals with functional impairment
A healthy lifestyle is important for patients with AAT deficiency. Health practices that can slow or prevent further lung damage are particularly important.
It is critical that you advise AAT deficient patients to stop smoking. Encouraging them to reduce their exposure to occupational and environmental pollutants, including second-hand tobacco smoke and all types of dust, is also important.
Patients should be strongly advised to stay current with immunizations, especially for influenza and pneumonia.
You also should encourage your patients to:
- Participate in a pulmonary rehabilitation plan
- Adhere to a good nutrition and exercise program
- Reduce alcohol consumption
- Develop a stress-management program
- Avoid exposure to people who are sick
- Maintain regular physician appointments and comply with prescribed medications and professional advice
Surgery for Advanced Disease
Lung transplantation can be considered for patients who do not respond to more conservative therapy or for those who have extensive lung damage.
Alpha-1 antitrypsin deficiency (AATD)
A genetic condition in which AAT is not released from the liver into the blood. As a result, the affected person does not have enough AAT throughout the body. Some individuals with AAT deficiency are not affected, but other individuals develop liver problems like cirrhosis and/or lung problems like emphysema.3
1. American Thoracic Society/European Respiratory Society Statement: Standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003; 168:818-900
2. Stoller JK, Rouhani R, Brantly M, et al. Biochemical efficacy and safety of a new pooled human plasma alpha1-antitrypsin, Respitin. Chest. 2002;122:66-74
3. American Lung Association. Alpha-1 Antitrypsin Deficiency Emphysema. Available at: http://www.lungusa.org/site/apps/nlnet/content3.aspx?c=dvLUK9O0E&b=4294229&ct=3004003 (Accessed September 18, 2008).