Bronchiectasis (Bx) is known to exist in both usual COPD and Alpha-1 Antitrypsin Deficiency (AATD) related COPD. However, the extent to which the two differ is unclear. We aimed to describe the current evidence for the characteristics of Bx in AATD.

Searches were performed for Bx and AATD on MEDLINE, EMBASE and Cochrane CENTRAL. Studies had to include detail of radiological findings, microbiology/microbiome or biochemical markers. No restrictions on outcomes were applied.

9 case reports, 3 case series, 3 case-control studies and 2 retrospective cohort studies were identified for a total of 751 patients; 322 had both Bx and AATD. Smoking and occupational histories were largely unrecorded. 14 articles studied radiology. Morphology was the most common descriptor: 61 patients (74.4%) had cylindrical, 14 (17.1%) varicose, 6 (7.3%) cystic and 1 (1.2%) traction Bx. Lobar distribution, airway thickening and severity were inconsistently reported. A standardised severity score was only used in one cohort.

4 articles (n=39 patients) described microbiology, with the most commonly reported organisms being P.aeruginosa, S.aureus, M.abscessus, and M.avium. Fungal cultures were positive in 29%. Microbiome data was unrecorded. 

2 articles studied serum biomarkers, with GC2 vitamin-D-binding-protein in AATD associating with Bx (OR 1.51), and IgA and IgG4 to pneumococcal vaccination higher in AATD and Bx than in AATD alone. Heterogeneity in exposure measures and outcomes, along with low quality, precluded further analysis.

Future research in this field should use standardised measures of radiological severity, and may include microbiome analysis.