August 21, 2025ERJ Open ResearchOpen Access

Characteristics of bronchiectasis in patients with different genotypes of severe alpha1-antitrypsin deficiency from the EARCO registry

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Bronchiectasis presents with distinct characteristics across AATD genotypes, particularly in disease severity and lung involvement patterns.
Among 349 patients, 70.5% were Pi*ZZ genotype, with worse lung function noted in rare genotypes and Pi*ZZ, FEV1 around 65.3%.
The analysis utilized data from the EARCO registry to examine CT findings and severity metrics like FACED and BSI scores.
Findings highlight a need for genotype-specific screening in AATD to enhance treatment strategies for affected individuals.

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Cross-sectional analysis reveals phenotypic differences in bronchiectasis severity among AATD genotypes, suggesting tailored management is crucial.

Mirizzi et al. (2025) studied this question.

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Cross-sectional analysis reveals phenotypic differences in bronchiectasis severity among AATD genotypes, suggesting tailored management is crucial.