How effective is IV augmentation therapy for the treatment of 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

Evidence for the use of alpha1-antitrypsin augmentation in patients after lung transplantation for alpha1-antitrypsin deficiency is insufficient. However, observational studies do show that inflammation from acute rejection or infection allows for free elastase activity in the epithelial lining fluid of individuals who have undergone lung transplantation. Therefore, the ATS/ERS Task Force favors the use of augmentation therapy for lung transplant recipients during episodes that provoke inflammation.

A 2008 commentary by the authors of the Medical and Scientific Advisory Committee of the Alpha-1 Foundation regarding the use of augmentation therapy for PI*MZ heterozygotes states that currently, until supportive data in the subset of heterozygotes becomes available, the only approved use for augmentation therapy is for PiZZ individuals. [19]

The Canadian Thoracic Society recommends the use of augmentation therapy in nonsmoking or smoking individuals with COPD attributable to emphysema and documented AATD who are receiving optimal and pharmacological and nonpharmacological therapy.


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