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α1-Antitrypsin

Hope on the Horizon for Emphysema Sufferers?

  • Review Article
  • Drug Therapy
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Summary

α1-Antitrypsin (α1AT) deficiency is the most common genetic cause of liver disease in children and emphysema in adults. Therapy for pulmonary disease attributable to α1AT deficiency includes α1AT augmentation therapy along with supportive measures. The α1AT preparation that is currently used for therapy is derived from fractionated plasma. The results of clinical trials suggest that augmentation therapy with α1AT slows the progression of emphysema and causes few adverse events. Patients with plasma levels of α1AT that are <11 μmol/L and who have airway obstruction should be considered for augmentation therapy. Novel approaches include the administration of aerosolised α1AT, recombinant α1AT, gene therapy and synthetic elastase inhibitors.

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  1. Section for Pulmonary Diseases, Department of Internal Medicine, Klinikum Grosshadern, University of Munich, Munich, Germany

    Martin Schwaiblmair & Claus Vogelmeier

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  1. Martin Schwaiblmair
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  2. Claus Vogelmeier
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Schwaiblmair, M., Vogelmeier, C. α1-Antitrypsin. Drugs & Aging 12, 429–440 (1998). https://doi.org/10.2165/00002512-199812060-00002

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