Progression and Augmentation Therapy in PiSZ and PiZZ Alpha-1 Antitrypsin Deficiency: A Longitudinal Functional and Densitometric Study
PiZZ individuals experience a more aggressive disease trajectory than PiSZ individuals in the absence of treatment. Augmentation therapy effectively mitigates disease progression in both genotypes, with greater efficacy when initiated early. Smoking and frequent exacerbations were identified as independent risk factors for accelerated disease progression. These findings underscore the importance of genotype-specific monitoring and personalized therapeutic strategies in AATD to optimize clinical outcomes.
Authors
- Juan Luis Rodríguez Hermosa (ORCID: https://orcid.org/0000-0003-0552-2484)
- Iman Esmaili (ORCID: https://orcid.org/0009-0004-5089-664X)
- Alvarez-Sala Jl (ORCID: https://orcid.org/0000-0002-5091-0286)
- Myriam Calle Rubio (ORCID: https://orcid.org/0000-0002-3890-2742)
- Gianna Vargas Centanaro
- Soha Esmaili (ORCID: https://orcid.org/0009-0007-3984-3557)
Institutions
- Universidad Complutense de Madrid (ES)
- Hospital Clínico San Carlos (ES)
- Instituto de Investigación Sanitaria del Hospital Clínico San Carlos (ES)
- Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (ES)
- 3v Geomatics (Canada) (CA)
Publication Details
- Journal
- Biomolecules
- Published
- 2025-04-17
- DOI
- https://doi.org/10.3390/biom15040599
- Primary Topic
- Protease and Inhibitor Mechanisms
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Sociedad Española de Neumología y Cirugía Torácica
- Grifols