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Alpha-1 Antitrypsin Deficiency Beyond COPD and Emphysema: A Narrative Review.
Lucia Pastoressa1, Vanessa Pivetti1, Marialuisa Valente1
1Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, 41124 Modena, Italy.
Medical Sciences (Basel, Switzerland)
|March 27, 2026
Summary
Alpha-1 antitrypsin deficiency (AATD) affects airways beyond emphysema, including bronchiectasis and asthma. Early diagnosis and targeted testing are crucial for managing this genetic disorder.
Area of Science:
- Pulmonology
- Genetics
- Immunology
Background:
- Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder typically linked to emphysema and COPD.
- Emerging evidence suggests AATD's spectrum includes airway diseases like bronchiectasis and asthma.
- Protease-antiprotease imbalance and neutrophilic inflammation are key mechanisms in AATD-associated airway injury.
Purpose of the Study:
- To review the relationship between AATD and airway diseases beyond emphysema.
- To focus on epidemiological patterns, mechanisms, diagnostics, and therapeutics.
- To synthesize current evidence on AATD in bronchiectasis, asthma, and severe asthma.
Main Methods:
- Narrative synthesis of literature.
- Integration of registry data, observational, and translational studies.
- Analysis of epidemiologic and mechanistic insights.
Main Results:
- AATD shows a notable prevalence in bronchiectasis and asthma, especially in severe or heterozygous genotypes.
- Shared mechanisms include neutrophil elastase overactivity, impaired mucociliary clearance, and neutrophilic inflammation.
- AATD in asthma is linked to T2-low, steroid-resistant phenotypes and persistent obstruction.
Conclusions:
- AATD is a systemic disorder with significant airway manifestations beyond COPD.
- Consider targeted AATD testing for idiopathic bronchiectasis and severe asthma.
- Further research is needed for causality, biomarkers, and anti-protease therapies.
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