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Comparison of outcomes in augmentation naïve and augmented patients with alpha-1 antitrypsin deficiency related lung disease

Paul Ellis, Kristen Holm, Radmila Choate, David Mannino, Robert Stockley, Robert Sandhaus, Alice Turner
European Respiratory Journal 2019 54: PA3383; DOI: 10.1183/13993003.congress-2019.PA3383
Paul Ellis
1Institute of Applied Health Research, University of Birmingham, Birmingham, United Kingdom
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  • For correspondence: p.ellis@bham.ac.uk
Kristen Holm
2Division of Medical, Behavioral, and Community Health, National Jewish Health, Denver, United States of America
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Radmila Choate
3Department of Preventive Medicine and Environmental Health, University of Kentucky College of Public Health, Lexington, United States of America
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David Mannino
3Department of Preventive Medicine and Environmental Health, University of Kentucky College of Public Health, Lexington, United States of America
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Robert Stockley
4Lung Investigation Unit Medicine, University Hospitals Birmingham NHS Foundation Trust Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
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Robert Sandhaus
5Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, United States of America
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Alice Turner
1Institute of Applied Health Research, University of Birmingham, Birmingham, United Kingdom
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Abstract

Alpha-1 antitrypsin (AAT) augmentation therapy is the only specific treatment available for alpha-1 antitrypsin deficiency (AATD) related lung disease. Randomised control trials (RCTs) have consistently demonstrated its benefit in preserving lung density as measured with CT densitometry. These studies were not adequately powered to detect differences in mortality or progression to lung transplant. Use of retrospective data may provide insight into effects of augmentation on survival and progression to lung transplant.

Comparison was made between augmentation naive AATD patients from the AATD UK registry (UK) and patients on augmentation therapy followed up in AlphaNet’s Disease Management and Prevention Program in the US.

Kaplan-Meier survival analysis of 1535 adult patients with severe AATD and associated lung disease was performed with matching for age, sex and smoking status. Time to death or lung transplant was taken from start of augmentation (treatment) or baseline assessment (control). Estimated mean survival was significantly longer in the treatment group: augmentation 20.3 years (95%CI 19.4 to 21.2), control 13.7 years (95%CI 13.1 to 14.3) p<0.001 (Figure 1).

This study suggests treatment with augmentation therapy prolongs survival or time to lung transplantation compared to those who are augmentation naïve and should be considered for AATD patients with lung disease.

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Footnotes

Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA3383.

This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).

  • Copyright ©the authors 2019
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Comparison of outcomes in augmentation naïve and augmented patients with alpha-1 antitrypsin deficiency related lung disease
Paul Ellis, Kristen Holm, Radmila Choate, David Mannino, Robert Stockley, Robert Sandhaus, Alice Turner
European Respiratory Journal Sep 2019, 54 (suppl 63) PA3383; DOI: 10.1183/13993003.congress-2019.PA3383

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Comparison of outcomes in augmentation naïve and augmented patients with alpha-1 antitrypsin deficiency related lung disease
Paul Ellis, Kristen Holm, Radmila Choate, David Mannino, Robert Stockley, Robert Sandhaus, Alice Turner
European Respiratory Journal Sep 2019, 54 (suppl 63) PA3383; DOI: 10.1183/13993003.congress-2019.PA3383
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