Asthma features in severe α1-antitrypsin deficiency: Experience of the National Heart, Lung, and Blood Institute registry

Edward Eden, Jeffrey Hammel, Farshid N. Rouhani, Mark L. Brantly, Alan Barker, A (Sonia) Buist, Robert J. Fallat, James K. Stoller, Ronald G. Crystal, Gerard M. Turino

Research output: Contribution to journalArticle

59 Citations (Scopus)

Abstract

Study objectives: To describe asthma features in a cohort with α1-antitrypsin (AAT) deficiency, and determine the impact of asthma on FEV1 decline. Background: Asthma may be common in those with AAT deficiency, and may lead to accelerated airflow obstruction. Design: Analysis of data obtained from a 5-year, prospective National Heart, Lung, and Blood Institute registry. Setting: A multicenter registry consisting of 37 clinical centers, a central phenotyping laboratory, and a data analysis center. Participants: A cohort of 1,052 subjects with AAT deficiency. Measurements and results: Asthma was defined as reversible airflow obstruction, recurrent attacks of wheezing, and a reported diagnosis of asthma or allergy with or without an elevated serum IgE level. FEV1 decline was calculated by least-square means with adjustments for covariables. Asthma was present in 21% of the cohort and in 12.5% of those with a normal FEV1. Attacks of wheezing were reported in 66%, the first attack occurring at a mean ± SD age of 31 ± 16 years. Allergy and asthma was reported in 29% and 38%, respectively. An elevated IgE level occurred in 17% and was significantly associated with signs and symptoms of asthma and an allergy history. Unadjusted FEV1 decline was less in the group without asthma and a normal IgE level (- 48.5 mL/yr) vs the groups with asthma features (≥ 64 mL/yr) [p = 0.002]. Multivariable analysis showed that bronchodilator response, age, and smoking were significant predictors for FEV1 decline but not asthma. Conclusions: Symptoms and signs of asthma are common in AAT deficiency and may start at the age of most rapid FEV1 loss. Adjusting for other risk factors such as bronchodilator response, asthma as defined does not lead to an accelerated FEV1 decline. In AAT deficiency, augmentation therapy is not more effective in preventing the loss of lung function in those with asthma compared to those without.

Original languageEnglish (US)
Pages (from-to)765-771
Number of pages7
JournalChest
Volume123
Issue number3
DOIs
StatePublished - Mar 1 2003

Fingerprint

National Heart, Lung, and Blood Institute (U.S.)
Registries
Asthma
Immunoglobulin E
Hypersensitivity
Bronchodilator Agents
Respiratory Sounds
Signs and Symptoms
Least-Squares Analysis

Keywords

  • α-antitrypsin deficiency
  • Asthma
  • Lung disease, obstructive

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Asthma features in severe α1-antitrypsin deficiency : Experience of the National Heart, Lung, and Blood Institute registry. / Eden, Edward; Hammel, Jeffrey; Rouhani, Farshid N.; Brantly, Mark L.; Barker, Alan; Buist, A (Sonia); Fallat, Robert J.; Stoller, James K.; Crystal, Ronald G.; Turino, Gerard M.

In: Chest, Vol. 123, No. 3, 01.03.2003, p. 765-771.

Research output: Contribution to journalArticle

Eden, E, Hammel, J, Rouhani, FN, Brantly, ML, Barker, A, Buist, AS, Fallat, RJ, Stoller, JK, Crystal, RG & Turino, GM 2003, 'Asthma features in severe α1-antitrypsin deficiency: Experience of the National Heart, Lung, and Blood Institute registry', Chest, vol. 123, no. 3, pp. 765-771. https://doi.org/10.1378/chest.123.3.765
Eden, Edward ; Hammel, Jeffrey ; Rouhani, Farshid N. ; Brantly, Mark L. ; Barker, Alan ; Buist, A (Sonia) ; Fallat, Robert J. ; Stoller, James K. ; Crystal, Ronald G. ; Turino, Gerard M. / Asthma features in severe α1-antitrypsin deficiency : Experience of the National Heart, Lung, and Blood Institute registry. In: Chest. 2003 ; Vol. 123, No. 3. pp. 765-771.
@article{09183d2a97674c0599fac029fe01d5eb,
title = "Asthma features in severe α1-antitrypsin deficiency: Experience of the National Heart, Lung, and Blood Institute registry",
abstract = "Study objectives: To describe asthma features in a cohort with α1-antitrypsin (AAT) deficiency, and determine the impact of asthma on FEV1 decline. Background: Asthma may be common in those with AAT deficiency, and may lead to accelerated airflow obstruction. Design: Analysis of data obtained from a 5-year, prospective National Heart, Lung, and Blood Institute registry. Setting: A multicenter registry consisting of 37 clinical centers, a central phenotyping laboratory, and a data analysis center. Participants: A cohort of 1,052 subjects with AAT deficiency. Measurements and results: Asthma was defined as reversible airflow obstruction, recurrent attacks of wheezing, and a reported diagnosis of asthma or allergy with or without an elevated serum IgE level. FEV1 decline was calculated by least-square means with adjustments for covariables. Asthma was present in 21{\%} of the cohort and in 12.5{\%} of those with a normal FEV1. Attacks of wheezing were reported in 66{\%}, the first attack occurring at a mean ± SD age of 31 ± 16 years. Allergy and asthma was reported in 29{\%} and 38{\%}, respectively. An elevated IgE level occurred in 17{\%} and was significantly associated with signs and symptoms of asthma and an allergy history. Unadjusted FEV1 decline was less in the group without asthma and a normal IgE level (- 48.5 mL/yr) vs the groups with asthma features (≥ 64 mL/yr) [p = 0.002]. Multivariable analysis showed that bronchodilator response, age, and smoking were significant predictors for FEV1 decline but not asthma. Conclusions: Symptoms and signs of asthma are common in AAT deficiency and may start at the age of most rapid FEV1 loss. Adjusting for other risk factors such as bronchodilator response, asthma as defined does not lead to an accelerated FEV1 decline. In AAT deficiency, augmentation therapy is not more effective in preventing the loss of lung function in those with asthma compared to those without.",
keywords = "α-antitrypsin deficiency, Asthma, Lung disease, obstructive",
author = "Edward Eden and Jeffrey Hammel and Rouhani, {Farshid N.} and Brantly, {Mark L.} and Alan Barker and Buist, {A (Sonia)} and Fallat, {Robert J.} and Stoller, {James K.} and Crystal, {Ronald G.} and Turino, {Gerard M.}",
year = "2003",
month = "3",
day = "1",
doi = "10.1378/chest.123.3.765",
language = "English (US)",
volume = "123",
pages = "765--771",
journal = "Chest",
issn = "0012-3692",
publisher = "American College of Chest Physicians",
number = "3",

}

TY - JOUR

T1 - Asthma features in severe α1-antitrypsin deficiency

T2 - Experience of the National Heart, Lung, and Blood Institute registry

AU - Eden, Edward

AU - Hammel, Jeffrey

AU - Rouhani, Farshid N.

AU - Brantly, Mark L.

AU - Barker, Alan

AU - Buist, A (Sonia)

AU - Fallat, Robert J.

AU - Stoller, James K.

AU - Crystal, Ronald G.

AU - Turino, Gerard M.

PY - 2003/3/1

Y1 - 2003/3/1

N2 - Study objectives: To describe asthma features in a cohort with α1-antitrypsin (AAT) deficiency, and determine the impact of asthma on FEV1 decline. Background: Asthma may be common in those with AAT deficiency, and may lead to accelerated airflow obstruction. Design: Analysis of data obtained from a 5-year, prospective National Heart, Lung, and Blood Institute registry. Setting: A multicenter registry consisting of 37 clinical centers, a central phenotyping laboratory, and a data analysis center. Participants: A cohort of 1,052 subjects with AAT deficiency. Measurements and results: Asthma was defined as reversible airflow obstruction, recurrent attacks of wheezing, and a reported diagnosis of asthma or allergy with or without an elevated serum IgE level. FEV1 decline was calculated by least-square means with adjustments for covariables. Asthma was present in 21% of the cohort and in 12.5% of those with a normal FEV1. Attacks of wheezing were reported in 66%, the first attack occurring at a mean ± SD age of 31 ± 16 years. Allergy and asthma was reported in 29% and 38%, respectively. An elevated IgE level occurred in 17% and was significantly associated with signs and symptoms of asthma and an allergy history. Unadjusted FEV1 decline was less in the group without asthma and a normal IgE level (- 48.5 mL/yr) vs the groups with asthma features (≥ 64 mL/yr) [p = 0.002]. Multivariable analysis showed that bronchodilator response, age, and smoking were significant predictors for FEV1 decline but not asthma. Conclusions: Symptoms and signs of asthma are common in AAT deficiency and may start at the age of most rapid FEV1 loss. Adjusting for other risk factors such as bronchodilator response, asthma as defined does not lead to an accelerated FEV1 decline. In AAT deficiency, augmentation therapy is not more effective in preventing the loss of lung function in those with asthma compared to those without.

AB - Study objectives: To describe asthma features in a cohort with α1-antitrypsin (AAT) deficiency, and determine the impact of asthma on FEV1 decline. Background: Asthma may be common in those with AAT deficiency, and may lead to accelerated airflow obstruction. Design: Analysis of data obtained from a 5-year, prospective National Heart, Lung, and Blood Institute registry. Setting: A multicenter registry consisting of 37 clinical centers, a central phenotyping laboratory, and a data analysis center. Participants: A cohort of 1,052 subjects with AAT deficiency. Measurements and results: Asthma was defined as reversible airflow obstruction, recurrent attacks of wheezing, and a reported diagnosis of asthma or allergy with or without an elevated serum IgE level. FEV1 decline was calculated by least-square means with adjustments for covariables. Asthma was present in 21% of the cohort and in 12.5% of those with a normal FEV1. Attacks of wheezing were reported in 66%, the first attack occurring at a mean ± SD age of 31 ± 16 years. Allergy and asthma was reported in 29% and 38%, respectively. An elevated IgE level occurred in 17% and was significantly associated with signs and symptoms of asthma and an allergy history. Unadjusted FEV1 decline was less in the group without asthma and a normal IgE level (- 48.5 mL/yr) vs the groups with asthma features (≥ 64 mL/yr) [p = 0.002]. Multivariable analysis showed that bronchodilator response, age, and smoking were significant predictors for FEV1 decline but not asthma. Conclusions: Symptoms and signs of asthma are common in AAT deficiency and may start at the age of most rapid FEV1 loss. Adjusting for other risk factors such as bronchodilator response, asthma as defined does not lead to an accelerated FEV1 decline. In AAT deficiency, augmentation therapy is not more effective in preventing the loss of lung function in those with asthma compared to those without.

KW - α-antitrypsin deficiency

KW - Asthma

KW - Lung disease, obstructive

UR - http://www.scopus.com/inward/record.url?scp=0037338319&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0037338319&partnerID=8YFLogxK

U2 - 10.1378/chest.123.3.765

DO - 10.1378/chest.123.3.765

M3 - Article

C2 - 12628876

AN - SCOPUS:0037338319

VL - 123

SP - 765

EP - 771

JO - Chest

JF - Chest

SN - 0012-3692

IS - 3

ER -