Association between α1-antitrypsin and bronchiectasis in patients with humoral immunodeficiency receiving gammaglobulin infusions

Brian P. Peppers, Jamie Zacharias, Chelsea R. Michaud, John A. Frith, Priya Varma, Mark Henning, Linda M. Quinn, Haig Tcheurekdjian, Timothy Craig, Robert W. Hostoffer

Research output: Contribution to journalArticle

Abstract

Background: In patients with humoral immunodeficiency, the progression of bronchiectasis has been known to occur despite adequate gammaglobulin therapy and in the absence of recurrent infections. This observation suggests that factors other than gammaglobulin replacement might play a part in the prevention of lung damage in this population. α1-Antitrypsin deficiency can be associated with bronchiectasis, a chronic inflammatory lung disease. The protective levels of α1-antitrypsin and phenotype in preventing bronchiectasis have not been thoroughly studied in the immunodeficient population. We hypothesized that patients with humoral immunodeficiencies on gammaglobulin infusions and bronchiectasis have lower median levels, but not necessary “classically” deficient levels, of α1-antitrypsin compared with those without bronchiectasis. Objective: To compare levels of α1-antitrypsin in subjects with immunodeficiency with and without bronchiectasis. Methods: One hundred ninety-two subjects with humoral immunodeficiencies requiring gammaglobulin therapy had their α1-antitrypsin levels and phenotype screened. High-resolution computed tomograms of the chest of participants were obtained and compared with α1-antitrypsin levels and phenotype. Results: Participants without bronchiectasis were found to have higher median levels of α1-antitrypsin than those with bronchiectasis (P =.003). Furthermore, subjects with improving or resolved bronchiectasis since initiating gammaglobulin therapy had higher median levels of α1-antitrypsin than those with worsening bronchiectasis (P =.004). The prevalence of the α1-antitrypsin PiZZ mutation was higher than in the general public (P <.0001). Conclusion: Median α1-antitrypsin levels and phenotype in subjects were associated with humoral immunodeficiency and their bronchiectasis status. Prospective studies might be necessary to determine possible benefits of augmentation therapy. This study supports the idea that what is considered a “normal or protective” α1-antitrypsin range might need to be refined for patients with humoral immunodeficiency on gammaglobulin therapy.

Original languageEnglish (US)
Pages (from-to)200-206
Number of pages7
JournalAnnals of Allergy, Asthma and Immunology
Volume120
Issue number2
DOIs
StatePublished - Feb 1 2018

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Bronchiectasis
Phenotype
Therapeutics
Population
Lung Diseases
Thorax
Prospective Studies

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology
  • Pulmonary and Respiratory Medicine

Cite this

Peppers, Brian P. ; Zacharias, Jamie ; Michaud, Chelsea R. ; Frith, John A. ; Varma, Priya ; Henning, Mark ; Quinn, Linda M. ; Tcheurekdjian, Haig ; Craig, Timothy ; Hostoffer, Robert W. / Association between α1-antitrypsin and bronchiectasis in patients with humoral immunodeficiency receiving gammaglobulin infusions. In: Annals of Allergy, Asthma and Immunology. 2018 ; Vol. 120, No. 2. pp. 200-206.
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title = "Association between α1-antitrypsin and bronchiectasis in patients with humoral immunodeficiency receiving gammaglobulin infusions",
abstract = "Background: In patients with humoral immunodeficiency, the progression of bronchiectasis has been known to occur despite adequate gammaglobulin therapy and in the absence of recurrent infections. This observation suggests that factors other than gammaglobulin replacement might play a part in the prevention of lung damage in this population. α1-Antitrypsin deficiency can be associated with bronchiectasis, a chronic inflammatory lung disease. The protective levels of α1-antitrypsin and phenotype in preventing bronchiectasis have not been thoroughly studied in the immunodeficient population. We hypothesized that patients with humoral immunodeficiencies on gammaglobulin infusions and bronchiectasis have lower median levels, but not necessary “classically” deficient levels, of α1-antitrypsin compared with those without bronchiectasis. Objective: To compare levels of α1-antitrypsin in subjects with immunodeficiency with and without bronchiectasis. Methods: One hundred ninety-two subjects with humoral immunodeficiencies requiring gammaglobulin therapy had their α1-antitrypsin levels and phenotype screened. High-resolution computed tomograms of the chest of participants were obtained and compared with α1-antitrypsin levels and phenotype. Results: Participants without bronchiectasis were found to have higher median levels of α1-antitrypsin than those with bronchiectasis (P =.003). Furthermore, subjects with improving or resolved bronchiectasis since initiating gammaglobulin therapy had higher median levels of α1-antitrypsin than those with worsening bronchiectasis (P =.004). The prevalence of the α1-antitrypsin PiZZ mutation was higher than in the general public (P <.0001). Conclusion: Median α1-antitrypsin levels and phenotype in subjects were associated with humoral immunodeficiency and their bronchiectasis status. Prospective studies might be necessary to determine possible benefits of augmentation therapy. This study supports the idea that what is considered a “normal or protective” α1-antitrypsin range might need to be refined for patients with humoral immunodeficiency on gammaglobulin therapy.",
author = "Peppers, {Brian P.} and Jamie Zacharias and Michaud, {Chelsea R.} and Frith, {John A.} and Priya Varma and Mark Henning and Quinn, {Linda M.} and Haig Tcheurekdjian and Timothy Craig and Hostoffer, {Robert W.}",
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Peppers, BP, Zacharias, J, Michaud, CR, Frith, JA, Varma, P, Henning, M, Quinn, LM, Tcheurekdjian, H, Craig, T & Hostoffer, RW 2018, 'Association between α1-antitrypsin and bronchiectasis in patients with humoral immunodeficiency receiving gammaglobulin infusions', Annals of Allergy, Asthma and Immunology, vol. 120, no. 2, pp. 200-206. https://doi.org/10.1016/j.anai.2017.11.014

Association between α1-antitrypsin and bronchiectasis in patients with humoral immunodeficiency receiving gammaglobulin infusions. / Peppers, Brian P.; Zacharias, Jamie; Michaud, Chelsea R.; Frith, John A.; Varma, Priya; Henning, Mark; Quinn, Linda M.; Tcheurekdjian, Haig; Craig, Timothy; Hostoffer, Robert W.

In: Annals of Allergy, Asthma and Immunology, Vol. 120, No. 2, 01.02.2018, p. 200-206.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Association between α1-antitrypsin and bronchiectasis in patients with humoral immunodeficiency receiving gammaglobulin infusions

AU - Peppers, Brian P.

AU - Zacharias, Jamie

AU - Michaud, Chelsea R.

AU - Frith, John A.

AU - Varma, Priya

AU - Henning, Mark

AU - Quinn, Linda M.

AU - Tcheurekdjian, Haig

AU - Craig, Timothy

AU - Hostoffer, Robert W.

PY - 2018/2/1

Y1 - 2018/2/1

N2 - Background: In patients with humoral immunodeficiency, the progression of bronchiectasis has been known to occur despite adequate gammaglobulin therapy and in the absence of recurrent infections. This observation suggests that factors other than gammaglobulin replacement might play a part in the prevention of lung damage in this population. α1-Antitrypsin deficiency can be associated with bronchiectasis, a chronic inflammatory lung disease. The protective levels of α1-antitrypsin and phenotype in preventing bronchiectasis have not been thoroughly studied in the immunodeficient population. We hypothesized that patients with humoral immunodeficiencies on gammaglobulin infusions and bronchiectasis have lower median levels, but not necessary “classically” deficient levels, of α1-antitrypsin compared with those without bronchiectasis. Objective: To compare levels of α1-antitrypsin in subjects with immunodeficiency with and without bronchiectasis. Methods: One hundred ninety-two subjects with humoral immunodeficiencies requiring gammaglobulin therapy had their α1-antitrypsin levels and phenotype screened. High-resolution computed tomograms of the chest of participants were obtained and compared with α1-antitrypsin levels and phenotype. Results: Participants without bronchiectasis were found to have higher median levels of α1-antitrypsin than those with bronchiectasis (P =.003). Furthermore, subjects with improving or resolved bronchiectasis since initiating gammaglobulin therapy had higher median levels of α1-antitrypsin than those with worsening bronchiectasis (P =.004). The prevalence of the α1-antitrypsin PiZZ mutation was higher than in the general public (P <.0001). Conclusion: Median α1-antitrypsin levels and phenotype in subjects were associated with humoral immunodeficiency and their bronchiectasis status. Prospective studies might be necessary to determine possible benefits of augmentation therapy. This study supports the idea that what is considered a “normal or protective” α1-antitrypsin range might need to be refined for patients with humoral immunodeficiency on gammaglobulin therapy.

AB - Background: In patients with humoral immunodeficiency, the progression of bronchiectasis has been known to occur despite adequate gammaglobulin therapy and in the absence of recurrent infections. This observation suggests that factors other than gammaglobulin replacement might play a part in the prevention of lung damage in this population. α1-Antitrypsin deficiency can be associated with bronchiectasis, a chronic inflammatory lung disease. The protective levels of α1-antitrypsin and phenotype in preventing bronchiectasis have not been thoroughly studied in the immunodeficient population. We hypothesized that patients with humoral immunodeficiencies on gammaglobulin infusions and bronchiectasis have lower median levels, but not necessary “classically” deficient levels, of α1-antitrypsin compared with those without bronchiectasis. Objective: To compare levels of α1-antitrypsin in subjects with immunodeficiency with and without bronchiectasis. Methods: One hundred ninety-two subjects with humoral immunodeficiencies requiring gammaglobulin therapy had their α1-antitrypsin levels and phenotype screened. High-resolution computed tomograms of the chest of participants were obtained and compared with α1-antitrypsin levels and phenotype. Results: Participants without bronchiectasis were found to have higher median levels of α1-antitrypsin than those with bronchiectasis (P =.003). Furthermore, subjects with improving or resolved bronchiectasis since initiating gammaglobulin therapy had higher median levels of α1-antitrypsin than those with worsening bronchiectasis (P =.004). The prevalence of the α1-antitrypsin PiZZ mutation was higher than in the general public (P <.0001). Conclusion: Median α1-antitrypsin levels and phenotype in subjects were associated with humoral immunodeficiency and their bronchiectasis status. Prospective studies might be necessary to determine possible benefits of augmentation therapy. This study supports the idea that what is considered a “normal or protective” α1-antitrypsin range might need to be refined for patients with humoral immunodeficiency on gammaglobulin therapy.

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