Abstract

Introduction: IMCA1 concluded that a poor outcome in patients with an AAT level <60mg/dl related to non-respiratory comorbidities more than to markers of respiratory disease1, but deaths only occurred in PiZZ patients.

Aims and Objectives: To assess outcome predictors in PiZZ (or equivalent genotype) patients.

Methods: All patients from participating centres during the initial phases of the pandemic were included. Backward stepwise logistic regression analysis was performed to identify variables related to poor outcomes, defined as hospitalization due to C-19 or death.

Results: 81 PiZZ patients were identified, 29 (36%) had a poor outcome, of whom 4 (5%) died. Regression analyses for variables associated with poor outcome and independent predictors of poor outcome are as tabulated.

Univariate Multivariate
Variable OR 95% CI p-value OR 95% CI p-value
Male sex 1.81 0.65-5.02 0.255
IV AAT augmentation 0.76 0.21-2.74 0.68
Emphysema on CT 2.53 0.75-8.53 0.133
Comorbidities 3.21 1.23-8.41 0.017 7.0 1.58-31.7 0.011
Hypertension 4.95 1.49-16.4 0.009
Worsening breathlessness 8.44 1.81-39.4 0.007 13.77 2.38-79.87 0.003
Anosmia 0.18 0.06-0.51 0.001
Dysgeusia 0.12 0.04-0.39 <0.001 0.04 0.01-0.19 <0.001
Age (yrs) 1.05 1.01-1.10 0.022
BMI (kg/m2) 0.99 0.9-1.08 0.759
O2 Sats (%) 0.93 0.86-1.00 0.058
FEV1%pred 0.97 0.96-0.99 0.007

Conclusions: Non-respiratory co-morbidities were more strongly related to poor outcome than biomarkers of respiratory disease. This relationship was more pronounced than in the wider group1, which had included patients with less severe deficiency (ie PiSZ and equivalent genotypes).

1Parr DG et al. Archivos de Bronconeumologia. 2022 12; 58(12):840-842.