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TL;DR
It found that bronchiectasis alone was present in 9.1% of patients, while 27% of those with emphysema also had bronchiectasis.
Orphanet Journal of Rare Diseases · 25 authors, 3 centres
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It found that bronchiectasis alone was present in 9.1% of patients, while 27% of those with emphysema also had bronchiectasis.
This registry-based cohort study analyzed data from the first 505 PiZZ genotype patients in the EARCO registry, of whom 418 had a baseline CT scan. The primary aim was to determine the prevalence of bronchiectasis and its characteristics. Key findings showed that 38 patients (9.1%) had bronchiectasis alone, and among the 303 patients with emphysema, 113 (27%) also had bronchiectasis. Patients with bronchiectasis alone or normal scans were predominantly female never smokers with near-normal lung function, while those with emphysema were older, more likely male smokers, and had moderate airflow obstruction and reduced gas transfer. The study has limitations, including the cross-sectional nature of the analysis, the fact that not all patients had CT scans, and that bronchiectasis morphology was not characterized in 42% of cases. The authors conclude that bronchiectasis is a significant feature in alpha-1 antitrypsin deficiency, present in nearly 10% of patients alone and in over a quarter of those with emphysema, warranting routine assessment and further clinical study.