A female patient with alpha-1 antitrypsin deficiency (AATD) was able to have a successful pregnancy and give birth to a healthy baby once receiving augmentation therapy, as published in Pulmonology.

The case report supports the initiation and use of augmentation therapy through intravenous injections of alpha-1 antitrypsin (AAT) during pregnancy. The patient began augmentation therapy at 18 weeks of pregnancy after her AAT levels had dropped from 74 mg/dL at 6 months prepregnancy to 38 mg/dL at 18 weeks. At 26 weeks of pregnancy and continued therapy, her AAT levels had increased to 108 mg/dL.

“This case adds to the very limited data regarding AAT therapy during pregnancy, supporting the argument that it can be safely initiated in response to severe impairment of respiratory function,” the authors said.


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The patient was initially diagnosed with AATD at age 21 years after a lack of response to treatment for severe asthma. She became pregnant at 29 years of age and had not initiated augmentation therapy prior to pregnancy. A moderate exacerbation of symptoms was experienced by the patient at weeks 17 and 18.

At week 18 the patient’s forced expiratory volume in the 1st second (FEV1) had decreased by 0.25 L compared to week 9 values, and because of the drop in FEV1 and AAT levels, the patient began augmentation therapy at week 18 at a dose of 60 mg/kg/week to maintain a blood threshold of 80 mg/dL.

The patient experienced a severe exacerbation at the 28th week of pregnancy and, after consultation, elected to have a caesarian section at week 32. Mild exertional dyspnea was reported at 4 weeks postpartum and a mild exacerbation was reported at 3 months follow-up.

In the 2 years after pregnancy, the patient only experienced 3 moderate exacerbations requiring oral antibiotics and/or corticosteroids and 1 severe exacerbation requiring hospitalization per year on average. This was a reduction compared to prepregnancy and augmentation therapy when the patient would average 10 moderate and 4 severe exacerbations per year.

“The case adds to the evidence that it is possible for patients with AATD to experience no pregnancy-related complications and deliver a healthy baby; however, close patient monitoring is essential to a positive outcome,” the authors concluded.

Reference

Guarnieri G, Achille A, Lococo S, Vianello A. Pregnancy in alpha 1 antitrypsin (AAT) deficiency and the role of intravenous AAT therapy. Pulmonology. Published online February 22, 2022. doi:10.1016/j.pulmoe.2022.01.014