Abstract
Introduction: Severe alpha1-antitrypsin (AAT) deficiency is associated with a high risk of airflow obstruction and emphysema. The risk of lung disease in those with intermediate AAT deficiency is unclear.
Aim: To assess pulmonary function, smoking habit, time of onset of symptoms, and indicators of quality of life (St. George Questionnaire) in patients with intermediate (PI*MZ) and severe (PI*ZZ) AATD in comparison with COPD patients without AATD (PI*MM).
Methods: Patients with PI*ZZ (n= 330), with PI*MZ (n=183), and with PI*MM (n= 100) with an established COPD diagnosis were recruited from the Italian Registry of Severe AATD. Functional, clinical, radiological, therapeutic, and quality of life data were collected.
Results: The three cohorts significantly differed in terms of age at diagnosis (P< 0.0001), respiratory function (FEV1 P= 0.001; FVC P= 0.001; DLCO P <0.001), quality of life (P= 0.0001) and smoking history (P <0.0001). At the multivariate analysis, ZZ genotype is significantly associated with a higher risk of airflow obstruction in comparison with MM [aOR= 44.9 (11.4-176.3); P <0.0001], showing a greater impact on obstruction than smoking history and age [ aOR= 5.9 (2.6-13.6); P <0.0001; aOR= 1.1(1.1-1.1)- P <0.0001, respectively]. The MZ genotype is not associated with a significant early risk of airflow obstruction.
Conclusion: The comparison of individuals with PI*ZZ, MZ, and MM genotypes can help assess the role of alpha1-antitrypsin deficiency on respiratory function and on the impact on quality of life.
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA3894.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019