Alpha-1-antitrypsin deficiency (AATD) is a hereditary condition requiring structured, evidence-based management. First-line care addresses modifiable risk factors alongside respiratory and nutritional needs to reduce disease progression.
First-line management begins with behavioural and pharmacological strategies targeting smoking cessation and avoidance of respiratory irritants. This is combined with preventive measures and a bronchodilator-based respiratory pharmacotherapy regimen.
Treatment for AATD includes behavioral and pharmacological therapy for smoking cessation, together with guidance on the importance of avoiding exposure to irritants (tobacco smoke, dust, smoke, pollutants or aerosolized chemicals).
Other therapies are similar to those indicated for patients with COPD not caused by AATD: anti-influenza and anti-pneumococcal vaccination; pharmacological treatment, as recommended by the GOLD, including the use of slow-release bronchodilators (muscarinic antagonists and β2 agonists) and inhaled corticosteroids (in special situations); pulmonary rehabilitation; and nutritional support.
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