Alpha1Health.com | Therapy for Alpha-1 Antitrypsin Deficiency | Emphysema and Alpha-1

Emphysema and Alpha-1

About Emphysema

Emphysema is a form of chronic obstructive pulmonary disease (COPD). Emphysema is caused by destruction of alveoli, small sac-like structures in the lungs where oxygen from the air is exchanged for carbon dioxide in the blood.

As a result of the damage, the fragile alveolar walls become filled with “holes.” Destruction of the alveolar walls leads to less surface area for gas exchange, which leads to less oxygen in the blood. The loss of elasticity also leads to air becoming trapped in the lungs, resulting in too much carbon dioxide in the blood.

Emphysema and Alpha-1

Individuals with alpha1-antitrypsin (AAT) deficiency* – also called Alpha-1 – are at risk for developing emphysema because of the unique role that AAT plays in protecting the lungs. An enzyme called neutrophil elastase (NE) normally is present in the lungs and plays a role in fighting bacteria. However, NE can destroy lung tissue if it is not neutralized by AAT.

*Also known as Alpha-1, AAT Deficiency, AATD, A1-PI deficiency, alpha-1 antiprotease deficiency, A1AD, or hereditary or genetic emphysema.

Individuals with alpha1-antitrypsin deficiency do not have enough AAT to maintain the balance between AAT and NE, and so have inadequate protection against the damaging effects of NE. 1

The symptoms and effects of emphysema are the same whether the emphysema is caused by AAT deficiency or by other factors, such as smoking. But there are several factors which differentiate typical emphysema from AAT-related emphysema.

Typical Emphysema

AAT-related Emphysema

Affects older individuals

Younger age of onset

Not associated with manifestations
outside the lungs

May be associated with liver
abnormalities and panniculitis

Usually associated with heavy
smoking history

May occur without smoking history
(Individuals with AAT deficiency who
do smoke may experience earlier
onset of symptoms.)

ARALAST [Alpha1-Proteinase Inhibitor (Human)] is indicated for chronic augmentation therapy in patients having congenital deficiency of A1-PI with clinically evident emphysema. ARALAST is not indicated as therapy for lung disease patients in whom congenital A1-PI deficiency has not been established.

Please review the Important Safety Information and the Full Prescribing Information

  1. American Lung Association. Alpha-1 Related Emphysema. Available at http://www.lungusa.org/site/pp.asp?c=dvLUK9O0E&b=35014#what . Accessed January 10, 2006.
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