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Alpha-1 Antitrypsin Deficiency

Alpha-1 Antitrypsin Deficiency (AATD or Alpha-1) is a genetic (inherited) condition – it is passed from parents to their children through their genes. Alpha-1 may result in serious lung disease in adults and/or liver disease at any age. Earlier screening helps to accurately diagnose Alpha-1, allowing for a treatment plan to be established.

Alpha-1 antitrypsin (A1AT) is a protein produced primarily by hepatocytes and released into circulation by the liver. A1AT functions in the lungs to inactivate neutrophil elastase, a powerful protease that contributes to the innate immune response.

A1AT keeps neutrophil elastase levels in check, thereby protecting the lung against alveolar wall destruction. A1AT provides > 90% of the anti-neutrophil elastase protection in the lungs.

AATD Information

respiratory overview 2A1AT deficiency (AATD) results in uninhibited neutrophil elastase activity, and this imbalance can lead to lung damage, including emphysema.

  • Caused by low levels of alpha1 antitrypsin (A1AT), a protein synthesized and secreted by the liver
  • A1AT protects the lungs from infection and lung diseases such as emphysema and COPD
  • More than 20 million individuals in the United States possess the genetically deficient alleles that cause AATD
  • Affects up to 5% of the approximately 12 million patients with COPD in the United States
  • An estimated 100,000 individuals are affected in the United States
  • An estimated 90% have not been diagnosed, and fewer are being treated
  • Lung-related symptoms usually develop between the ages of 20 and 40



Earlier screening helps to accurately diagnose Alpha-1, allowing for a treatment plan to be established that may lead to improved outcomes.
Lifestyle changes such as smoking cessation, healthy eating habits, and regular exercise can improve overall health.
A1AT augmentation therapy has been shown to raise and maintain serum levels of A1AT protein.

The Journal of the COPD Foundation states intravenous augmentation therapy in those with AATD is recommended for:

  • Individuals with an FEV1 less than or equal to 65% predicted.
  • For those with lung disease related to AATD and an FEV1 greater than 65%, we recommend discussion of potential benefits of reducing lung function decline with consideration of the cost of therapy and lack of evidence for such benefit.
  • Individuals with necrotizing panniculitis.
For U.S. Healthcare Professionals only
For U.S. Healthcare Professionals only

The purpose of this CSL Behring Medical Affairs website is to support Healthcare Professionals with scientific information. This website is also a channel for U.S. Healthcare Professionals to submit questions or connect with CSL Behring U.S. Healthcare Professionals. The information provided is for educational purposes only and is not intended to promote any products. By continuing to use this site you are acknowledging that you are a US Healthcare Professional

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