What are the recommendations for the use of 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

Current guidelines recommend augmentation therapy for individuals with abnormal alpha1-antitrypsin genotypes who have alpha1-antitrypsin levels below 11 μM and documented evidence of airflow obstruction in pulmonary function tests. [16, 17]

While no firm guidelines have been developed for initiating or continuing augmentation therapy, most pulmonary physicians require the serum level to be below the threshold protective value and that the patient have one or more of the following: signs of significant lung disease: chronic productive cough or unusual frequency of lower respiratory infection, airflow obstruction, accelerated decline of FEV1, or chest radiographic or CT evidence of emphysema.

The ATS recommends starting treatment when the FEV1 is less than 80% of the patient's predicted value, though the benefits of augmentation therapy for individuals with severe (FEV1<35%) or mild (FEV1 >60%) airflow obstruction are less, as shown in studies with Prolastin.


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