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

Int J Chron Obstruct Pulmon Dis. 2018 Mar 23;13:1001-1007. doi: 10.2147/COPD.S155226. eCollection 2018.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Chi-Square Distribution
  • Disease Progression
  • Female
  • Forced Expiratory Volume
  • Humans
  • Linear Models
  • Lung / physiopathology*
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Phenotype
  • Pneumonia / complications
  • Prognosis
  • Pulmonary Emphysema / diagnosis
  • Pulmonary Emphysema / etiology*
  • Registries
  • Retrospective Studies
  • Risk Factors
  • Smoking / adverse effects
  • Spain
  • Spirometry
  • Time Factors
  • alpha 1-Antitrypsin Deficiency / complications*
  • alpha 1-Antitrypsin Deficiency / diagnosis
  • alpha 1-Antitrypsin Deficiency / physiopathology