%0 Journal Article
%T Predicting Exacerbations in Alpha-1 Antitrypsin Deficiency Using Clinical and Pulmonary Function Tests: Portuguese EARCO Registry.
%A Faria N
%A Gomes J
%A Guimarães C
%A Marçôa R
%A Ferraz B
%A Sucena M
%J Respiration
%V 103
%N 6
%D 2024 Mar 26
%M 38531325
%F 3.966
%R 10.1159/000537759
%X <B>BACKGROUND: </B>Exacerbations are common in individuals with alpha-1 antitrypsin deficiency (AATD)-related lung disease. This study intended to identify independent predictive factors for exacerbations in AATD using the Portuguese European Alpha-1 Research Collaboration (EARCO) registry.<BR><B>METHODS: </B>This study includes patients from the Portuguese EARCO registry, a prospective multicenter cohort (NCT04180319). From October 2020 to April 2023, this registry enrolled 137 patients, 14 of whom were excluded for analysis for either missing 12 months of follow-up or baseline pulmonary function.<BR><B>RESULTS: </B>Among the 123 AATD patients, 27 (22.0%) had at least one exacerbation in the last 12 months of follow-up. Patients with Pi*ZZ phenotype were three times more likely than the rest of the population to experience any exacerbation (32.7 vs. 14.1%, p = 0.014; OR 3.0). BODE index was significantly higher in exacerbators than in non-exacerbators (3.9 ± 2.4 vs. 1.3 ± 1.2; p &lt; 0.001), including on multivariate analysis (p = 0.002). Similar results were found for BODEx (multivariate p &lt; 0.001). DLCO was the only functional parameter independently associated with exacerbations (p = 0.024).<BR><B>CONCLUSIONS: </B>DLCO, BODE, and BODEx were independent predictors of exacerbations at 12 months in AATD patients. Understanding these risk factors can aid decision-making on AATD-related lung disease management and improve patient outcomes.