How is lung function improved in patients with alpha1-antitrypsin deficiency (AATD)?

Updated: Sep 11, 2020
  • Author: Dora E Izaguirre Anariba, MD, MPH; Chief Editor: John J Oppenheimer, MD  more...
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Answer

Provide similar efforts to improve lung function in patients with AATD emphysema as those provided to patients with emphysema from the usual causes.

Administer short-acting beta-adrenergic agents and ipratropium bromide bronchodilators to maximize lung function. Metered-dose inhalers are the preferred method of administration because they have a lower incidence of adverse effects than other routes. No matter how they are administered, no evidence indicates that these drugs have any long-term effect on disease progression.

Inhaled corticosteroids have not been studied in patients with AATD emphysema, but many patients have significant bronchoreactivity. In this group, inhaled steroids probably help control symptoms. Patients with frequent exacerbations may also benefit. Evidence of infection can be an adverse effect.

Long-acting inhaled beta-adrenergic drugs and anticholinergics provide improved bronchodilation and symptoms for patients with COPD. They have not been studied in a population with AATD, but they are likely to provide the same benefits.

Reserve oral corticosteroids for acute exacerbations with increased cough and sputum. Long-term administration of corticosteroids does not protect the lung from progressive emphysema, but it is associated with many detrimental adverse effects. Limit oral steroid use to brief courses of 1-2 weeks. Start therapy to prevent osteoporosis when long courses are administered.

Theophylline may lessen the degree of dyspnea in some individuals, and a therapeutic trial may be indicated for selected patients. The therapeutic range of theophylline is relatively small, and its metabolism frequently is altered by other drugs or illness, which can lead to frequent episodes of drug toxicity or the need for frequent monitoring of serum levels. It also should be noted that theophylline is metabolized by the liver. Likewise, when smoking, metabolism is actually increased; thus, smoking cessation may effect levels.


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