Bronchiectasis but not emphysema is more prevalent in non-PiZ alpha-1 antitrypsin deficiency (AATD) COPD than in usual COPD

摘要: Bronchiectasis (BE) is associated with PiZ phenotypes of AATD. An association non-PiZ (SS, MS, MZ) less certain. We wished to conduct a case-control study BE prevalence among SS, MS and MZ genotypes vs. usual COPD. 16 subjects (NZ) (3 8 5 from community COPD screening for AATD had undergone chest CT scans. They were compared age, sex FEV 1 matched patients AAT levelsl1.15g/L but MM genotype (MM) similarly serum levels≥1.15g/L not genotyped (NG). Spirometry, lung volumes diffusing capacity (D Lco ) measured within 5.9±5.2SD months imaging. scans read using visual grading system. Lung emphysema on scan was determined Airway Inspector (www.airwayinspector.org) measure %areal-950HU (LAA%). Group differences between continuous variables ANOVA proportions z-statistic. Gender M30:F18, age 70±18 years (mean±SD), smoking history 47±26 pack-years, 68±23 %predicted /FVC 55±13 % in the 48 subjects. Serum levels NZ 0.89±0.01SE g/L, 0.90±0.05 NG 1.59±0.07. occurred 13/16 (3/3 6/8 4/5 MZ), 4/16 (p=0.00071) 5/16 (p=0.00219). Groups similar spirometry, D . LAA% did differ groups (NZ 12.3±3.7SE, 12.0±2.7, 11.0±3.2, p=0.97). In this small group patients, present more often phenotype than subjects, or normal levels. This may be novel observation requiring confirmation larger populations.