Patient management

The management of alpha-1-antitrypsin (A1AT) deficiency (also known as alpha-1) and associated obstructive pulmonary disease is based on current recommendations from the American Thoracic Society/European Respiratory Society1 and the Global Initiative for Chronic Obstructive Lung Disease.2 The approach is multimodal and includes both nonpharmacologic and pharmacologic components.

Nonpharmacologic Recommendations

  • Avoidance of smoking1
  • Avoidance of exposure to chemicals that can cause respiratory irritation1
  • Use of supplemental oxygen when indicated (eg, during air travel)1
  • Regular physical activity2
  • Nutritional support or supplementation, if needed2
  • Pulmonary rehabilitation in patients with functional impairment1
  • Consider lung transplantation in carefully selected patients with severe functional impairment and airflow obstruction1

Pharmacologic recommendations

  • Appropriate vaccination for influenza and pneumococcus1
  • Use of inhaled bronchodilators1
  • Appropriate management of acute exacerbations, including use of systemic corticosteroids, antibiotics, and noninvasive ventilator support if needed1
  • Intravenous administration of A1AT (or augmentation therapy), in patients with established airflow obstruction attributable to severe A1AT deficiency1
 

References

  1. American Thoracic Society/European Respiratory Society statement: standards for the diagnosis and management of individuals with alpha-1 antitrypsin deficiency. Am J Respir Crit Care Med. 2003;168:818-900.
  2. Global Initiative for Chronic Obstructive Lung Disease. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. 2016. http://goldcopd.org/global-strategy-diagnosis-management-prevention-copd-2016/. Accessed July 14, 2016.

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