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Long-term evolution of lung function in individuals with alpha-1 antitrypsin deficiency from the Spanish registry (REDAAT)

Authors Esquinas C, Serreri S, Barrecheguren M, Rodríguez E, Nuñez A, Casas-Maldonado F, Blanco I, Pirina P, Lara B, Miravitlles M

Received 26 October 2017

Accepted for publication 29 January 2018

Published 23 March 2018 Volume 2018:13 Pages 1001—1007

DOI https://doi.org/10.2147/COPD.S155226

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Charles Downs

Peer reviewer comments 3

Editor who approved publication: Dr Richard Russell


Cristina Esquinas,1,2,* Sonia Serreri,3,* Miriam Barrecheguren,1 Esther Rodriguez,1 Alexa Nuñez,1 Francisco Casas-Maldonado,4 Ignacio Blanco,5 Pietro Pirina,3 Beatriz Lara,6 Marc Miravitlles1,7

1Pneumology Department, University Hospital Vall d’Hebron, Barcelona, Spain; 2Public Health, Mental, Maternal and Child Health Nursing Department, Faculty of Medicine and Health Sciences, University of Barcelona, Barcelona, Spain; 3Università di Sassari, Sassari, Italy; 4Pneumology Department, University Hospital San Cecilio, Granada, Spain; 5Alpha-1 Antitrypsin Deficiency Spanish Registry (REDAAT), Spanish Society of Pneumology (SEPAR), Barcelona, Spain; 6Coventry and Warwickshire University Hospital, Coventry, UK; 7CIBER de Enfermedades Respiratorias (CIBERES), Spain

*These authors contributed equally to this work

Background:
The clinical course of alpha-1 antitrypsin deficiency (AATD) is very heterogeneous. It is estimated that 60% of individuals with severe AATD (Pi*ZZ) develop emphysema. The main objective of this study was to describe the outcomes of long-term lung function in individuals with AATD-associated emphysema after at least 8 years of follow-up.
Materials and methods: We performed a retrospective analysis of longitudinal follow-up data of AATD PiZZ patients from the Spanish registry (AATD Spanish Registry [REDAAT]). The main follow-up outcome was the annual rate of decline in forced expiratory volume in 1 second (FEV1) calculated using the FEV1 values at baseline and in the last post-bronchodilator spirometry available.
Results: One hundred and twenty-two AATD PiZZ patients were analyzed. The median follow-up was 11 years (interquartile range =9–14). The mean FEV1 decline was 28 mL/year (SD=54), with a median of 33 mL/year. Tobacco consumption (β=19.8, p<0.001), previous pneumonia (β=27.8, p=0.026) and higher baseline FEV1% (β=0.798, p=0.016) were independently related to a faster FEV1 decline.
Conclusion: In this large cohort with a long follow-up, we observed a very variable decline of FEV1. However, the mean FEV1 decline was similar to that observed in large cohorts of smoking-related COPD. Tobacco consumption, previous pneumonia and better lung function at baseline were related to a faster decline in FEV1. These results highlight the importance of early diagnosis and effective treatment.

Keywords: alpha-1 antitrypsin deficiency, lung function, registers

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