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Nonspecific treatment for alpha1-antitrypsin (AAT) deficiency includes modalities commonly used for all cases of COPD, such as:
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Smoking cessation |
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Bronchodilators |
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Pneumococcal and influenza vaccination |
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Corticosteroids |
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Modest exercise |
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Oxygen supplementation |
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Pulmonary rehabilitation |
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Improved nutrition |
Based on data from PiSZ individuals (who rarely develop premature emphysema), it appears that serum AAT levels ≥11 micromoles (μM) are protective against accelerated lung destruction.1 Early attempts at raising AAT serum levels included the administration of the anabolic steroid danazol2 or tamoxifen.3,4 It was postulated that these agents might induce an acute-phase response with increased AAT synthesis. However, although AAT levels in plasma and epithelial lining fluid (ELF) increased, they remained below the protective threshold in PiZ patients. Furthermore, the expected adverse events with these potential treatments limit their long-term use.
The most direct, logical, and specific therapeutic approach to this inherited lung disease is augmentation therapy with AAT to maintain plasma levels ≥11 micromoles μM.
References:
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Turino GM, Barker AF, Brantly ML, et al. Clinical features of individuals with PiSZ phenotype of alpha1-antitrypsin deficiency. Am J Resp Crit Care Med. 1996;154:1718-1725. |
| 2. |
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Gadek JE, Fulmer JD, Gelfand JA, et al. Diazole-induced augmentation of serum alpha1-antitrypsin levels in individuals with marked deficiency of this antiprotease. J Clin Invest. 1980;66:82-87. |
| 3. |
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Eriksson S. The effect of tamoxifen in intermediate alpha1-antitrypsin deficiency associated with the phenotype PiSZ. Ann Clin Res. 1983;15:95-98. |
| 4. |
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Wewers MD, Brantly ML, Casolaro MA, Crystal RG. Evaluation of tamoxifen as a therapy to augment alpha1-antitrypsin concentrations in Z homozygous alpha1-antitrypsin deficient subjects. Am Rev Respir Dis. 1987;135:401-402. |
Important Safety Information
Prolastin®, Alpha1-Proteinase Inhibitor (Human) is indicated for chronic replacement therapy of individuals having congenital deficiency of alpha-1 PI (alpha1-antitrypsin deficiency) with clinically demonstrable panacinar emphysema. Weekly Prolastin® therapy has demonstrated a low occurrence of side effects. In clinical studies with Prolastin®, reactions were observed in 1.16% of infusions, the most common events being fever (0.77%), light-headedness (0.19%), and dizziness (0.19%). As with all plasma-derived therapeutics, the potential to transmit infectious agents cannot be totally eliminated. Individuals with selective IgA deficiencies who have known antibody against IgA (anti-IgA antibody) should not receive Prolastin®, since these patients may experience severe reactions, including anaphylaxis, to IgA which may be present.
You are encouraged to report negative side effects of prescription drugs
to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.
Please click here for Prolastin® full Prescribing Information.
Remember, your doctor or healthcare provider is the single best source of information regarding you and your health. Please consult your doctor or healthcare provider if you have any questions about your health or any of your medications.
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