PMID- 38531325
OWN - NLM
STAT- Publisher
LR  - 20240326
IS  - 1423-0356 (Electronic)
IS  - 0025-7931 (Linking)
DP  - 2024 Mar 26
TI  - Predicting exacerbations in alpha1-antitrypsin deficiency using clinical and 
      pulmonary function tests: Portuguese EARCO registry.
LID - 10.1159/000537759 [doi]
AB  - 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 multicentre 
      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.
CI  - S. Karger AG, Basel.
FAU - Faria, Nuno
AU  - Faria N
FAU - Gomes, Joana
AU  - Gomes J
FAU - Guimaraes, Catarina
AU  - Guimaraes C
FAU - Marcoa, Raquel
AU  - Marcoa R
FAU - Ferraz, Beatriz
AU  - Ferraz B
FAU - Sucena, Maria
AU  - Sucena M
LA  - eng
PT  - Journal Article
DEP - 20240326
PL  - Switzerland
TA  - Respiration
JT  - Respiration; international review of thoracic diseases
JID - 0137356
SB  - IM
EDAT- 2024/03/27 00:44
MHDA- 2024/03/27 00:44
CRDT- 2024/03/26 19:24
PHST- 2023/11/03 00:00 [received]
PHST- 2024/02/05 00:00 [accepted]
PHST- 2024/03/27 00:44 [medline]
PHST- 2024/03/27 00:44 [pubmed]
PHST- 2024/03/26 19:24 [entrez]
AID - 000537759 [pii]
AID - 10.1159/000537759 [doi]
PST - aheadofprint
SO  - Respiration. 2024 Mar 26. doi: 10.1159/000537759.