Introduction: The relationship between alpha 1 antitrypsin deficiency (AATD) and bronchiectasis (BC) is well established, however not much is known about AATD progression in patients with BC.

Aims and objectives: To study the impact of BC on the disease progression in patients with severe AATD.

Methods: Cross-sectional study, including all patients diagnosed with severe AATD in two tertiary hospitals from 2013 to 2020.

Results: 55 patients (34 men, mean age of 65.45 years) were analyzed and divided into two groups according to radiological evidence of BC (27 patients with BC, 49.1%); mean A1AT levels were 54.24(±26.09) mg/dL with no statistical difference between groups (p>0.05). 
 
The average number of exacerbations per year was 0.96 in the BC group and 0.71 in the non-BC group (p=0.29).
Only 43 patients had a follow-up lung function test (LFT) with a 2-year gap. Initial LFT was comparable between groups (p>0.05). The decline in FEV1 and FVC between the 2 groups was compared; FVC in patients with BC decreased 0.03L and without BC 0.08L. FEV1 in patients with BC decreased by 0.012L and without BC by 0.002L. None of these differences was statistically significant. 
Dyspnea was more severe in patients with BC (mean mMRC=1.37(±1.12) points) than without BC (mMRC=1.25(±1.24) points)(p=0.048).
4 patients were under A1AT augmentation therapy, 3 of them with BCs.

Conclusion: Although only dyspnea severity showed a statistically significant difference between the two groups, patients with BC were more symptomatic, had a higher number of exacerbations and a greater lung function decline than patients without BC.
We believe that BC group of patients should be maintained in a closer follow-up.