What Is AAT Deficiency?

Alpha1-antitrypsin (AAT) deficiency (also known as Alpha-1) develops when your body has a lower than normal level of the protein AAT. It is a genetic disorder, so it is inherited.2 Because Alpha patients have low levels of the protective protein called AAT in their blood, certain enzymes are allowed to attack healthy tissues in the body, primarily in the lungs. Such destruction can lead to serious lung disease (such as emphysema), liver conditions, and skin conditions.


AAT plays a critical role in protecting your lungs1

  • View All
  • Neutrophils

  • Normal AAT levels

  • Low levels of AAT

Neutrophils

Neutrophils mobilize, delivering elastase at the alveolar surface in response to bacterial infections, environmental pollutants, or tobacco exposure.

Normal AAT levels keep neutrophil elastase in check. AAT inhibits excess elastase; lung structure preserved.

Low levels of AAT leave lung tissue unprotected. Excess elastase cannot be neutralized; lung elastin destroyed; lung function compromised.

Neutrophils

Neutrophils mobilize, delivering elastase at the alveolar surface in response to bacterial infections, environmental pollutants, or tobacco exposure.

Normal AAT levels

Normal AAT levels keep neutrophil elastase in check. AAT inhibits excess elastase; lung structure preserved.

Low levels of AAT

Low levels of AAT leave lung tissue unprotected. Excess elastase cannot be neutralized; lung elastin destroyed; lung function compromised.



TESTING FOR ALPHA-1 IS IMPORTANT

Getting tested for alpha-1 is easy. It is diagnosed with simple blood tests. Find out about a free Grifols AlphaKit you can get from your doctor.



PROLASTIN®-C (alpha1-proteinase inhibitor [human]) is indicated for chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to severe hereditary deficiency of alpha1-PI (alpha1-antitrypsin deficiency).

The effect of augmentation therapy with any alpha1-proteinase inhibitor (alpha1-PI), including PROLASTIN-C, on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been conclusively demonstrated in randomized, controlled clinical trials. Clinical data demonstrating the long-term effects of chronic augmentation or maintenance therapy with PROLASTIN-C are not available.

PROLASTIN-C is not indicated as therapy for lung disease in patients in whom severe alpha1-PI deficiency has not been established.

PROLASTIN-C is contraindicated in IgA-deficient patients with antibodies against IgA due to the risk of severe hypersensitivity and in patients with a history of anaphylaxis or other severe systemic reactions to alpha1-PI.

Hypersensitivity reactions, including anaphylaxis, may occur. Monitor vital signs and observe the patient carefully throughout the infusion. Should hypersensitivity symptoms be observed, promptly stop infusion and begin appropriate therapy. Have epinephrine and other appropriate therapy available for the treatment of any acute anaphylactic or anaphylactoid reaction.

PROLASTIN-C may contain trace amounts of IgA. Patients with known antibodies to IgA, which can be present in patients with selective or severe IgA deficiency, have a greater risk of developing potentially severe hypersensitivity and anaphylactic reactions.

The most common drug-related adverse reaction observed at a rate of >5% in subjects receiving PROLASTIN-C was upper respiratory tract infection. The most serious adverse reaction observed during clinical trials with PROLASTIN-C was an abdominal and extremity rash in 1 subject.

Because PROLASTIN-C is made from human plasma, it may carry a risk of transmitting infectious agents, eg, viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent. This also applies to unknown or emerging viruses and other pathogens.

Please click here for full Prescribing Information for PROLASTIN-C.


References

  1. Köhnlein T, Welte T. Alpha-1 Antitrypsin Deficiency: Clinical Aspects and Management. Bremen, Germany: Uni-Med Verlag AG; 2007:16-39.
  2. What is Alpha-1? Alpha-1 Foundation. http://www.alphaone.org/healthcare/?c=01.what is alpha-1