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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.


Alpha 1 antitrypsin (A1-PI) treatment slows emphysema progression independent of baseline FEV1

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Please see full prescribing information for ZEMAIRA

Effect of exacerbations on lung density in alpha-1

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Please see full prescribing information for ZEMAIRA

The effect of exacerbations on lung density in relation to patient characteristics in the RAPID-RCT trial of alpha-1 antitrypsin therapy

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Please see full prescribing information for ZEMAIRA

Key Publications

Important Safety Information for ZEMAIRA

Alpha1-Proteinase Inhibitor (Human), ZEMAIRA® is contraindicated in patients with a history of severe systemic reactions to the product or to A1-PI protein, including anaphylaxis. Due to the risk of severe hypersensitivity, ZEMAIRA is also contraindicated in immunoglobulin A-deficient patients with antibodies against IgA.

Use caution in administering ZEMAIRA to patients who have experienced anaphylaxis or severe systemic reactions to another A1-PI product. Patients with selective or severe IgA deficiency can develop antibodies to IgA and are at greater risk of such reactions. If anaphylactic or severe anaphylactoid reactions occur during infusion, discontinue immediately.

In pre-licensure clinical studies, the following adverse reactions were reported in at least 5% of subjects receiving ZEMAIRA: headache, sinusitis, upper respiratory infection, bronchitis, asthenia, increased cough, fever, injection-site hemorrhage, rhinitis, sore throat, and vasodilation.

ZEMAIRA is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent and its variant (vCJD), cannot be completely eliminated.

Indications for ZEMAIRA

ZEMAIRA is indicated for chronic augmentation and maintenance therapy for adults with alpha1-proteinase inhibitor (A1-PI) deficiency and emphysema. The effect of augmentation therapy with ZEMAIRA or any A1-PI product on pulmonary exacerbations and progression of emphysema in A1-PI deficiency has not been demonstrated in randomized, controlled clinical studies.

ZEMAIRA is not indicated for lung disease patients in whom severe A1-PI deficiency has not been established.

Please see full prescribing information for ZEMAIRA.

To report SUSPECTED ADVERSE REACTIONS, contact the CSL Behring Pharmacovigilance Department at 1-866-915-6958 or FDA at 1-800-FDA-1088 or

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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