1. Predicting Exacerbations in Alpha-1 Antitrypsin Deficiency Using Clinical and Pulmonary Function Tests: Portuguese EARCO Registry.
- Author
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Faria, Nuno, Gomes, Joana, Guimarães, Catarina, Marçôa, Raquel, Ferraz, Beatriz, and Sucena, Maria
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DISEASE exacerbation , *PULMONARY function tests , *RISK assessment , *SECONDARY analysis , *PULMONARY gas exchange , *QUESTIONNAIRES , *REPORTING of diseases , *DESCRIPTIVE statistics , *MULTIVARIATE analysis , *ODDS ratio , *LUNG diseases , *ALPHA 1-antitrypsin deficiency , *PHENOTYPES , *DISEASE risk factors , *DISEASE complications - Abstract
Introduction: 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. Methods: 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. Results: 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 < 0.001), including on multivariate analysis (p = 0.002). Similar results were found for BODEx (multivariate p < 0.001). DLCO was the only functional parameter independently associated with exacerbations (p = 0.024). Conclusions: 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. [ABSTRACT FROM AUTHOR]
- Published
- 2024
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