Demographic and clinical characteristics of patients with α 1 -antitrypsin deficiency genotypes PI*ZZ and PI*SZ in the Spanish registry of EARCO.

There is a high prevalence of PI*SZ in Spain. Patients with the PI*SZ genotype were older at symptom onset and diagnosis and had less severe lung disease compared with PI*ZZ patients. The prevalence of asthma was higher in PI*SZ, and up to 15% of PI*SZ patients received augmentation therapy.