Exacerbations in subjects with alpha-1 antitrypsin deficiency receiving augmentation therapy

Michael A Campos, Saleh Alazemi, Guoyan Zhang, Adam Wanner, Matthias A Salathe, Horst Baier, Robert A. Sandhaus

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Background: The frequency, characteristics and impact of acute exacerbations in patients with alpha-1 antitrypsin deficiency (AATD) and COPD who are on intravenous alpha-1 antitrypsin augmentation therapy have not been described. Methods: 922 subjects with AATD and COPD on augmentation therapy (mean age 54.5 years) were followed with monthly telephone surveys to record exacerbation characteristics, as well as healthcare resource utilization and health-related quality of life (HRQoL). Exacerbations were defined by symptom-based and healthcare resource utilization (HRU) criteria. Results: During the 1-year follow-up, 91.5% of participants experienced at least one exacerbation (mean 2.4 exacerbations per subject, median 2, and mean duration 17 days per episode, regardless of the definition used). Most exacerbations were categorized as severe by symptoms and moderate by HRU criteria. Subjects who had 3 or more exacerbations (48.6%) were younger, had higher medication use and had higher tobacco consumption compared with subjects with less exacerbations. Subjects with frequent exacerbations had the worst baseline HRQoL scores, as well as more physician visits, emergency room visits, and hospitalizations. Although most subjects received augmentation therapy on a weekly basis, other infusion schedules were more commonly observed in subjects with fewer exacerbations. Conclusion: COPD exacerbations occur frequently and are associated with significant disease burden in subjects with AATD receiving augmentation therapy.

Original languageEnglish
Pages (from-to)1532-1539
Number of pages8
JournalRespiratory Medicine
Volume103
Issue number10
DOIs
StatePublished - Oct 1 2009

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alpha 1-Antitrypsin Deficiency
Chronic Obstructive Pulmonary Disease
Delivery of Health Care
Quality of Life
alpha 1-Antitrypsin
Tobacco Use
Therapeutics
Telephone
Hospital Emergency Service
Appointments and Schedules
Hospitalization
Physicians
Autosomal Recessive alpha-1-Antitrypsin Deficiency

Keywords

  • Alpha-1 antitrypsin deficiency
  • Exacerbations
  • Health-related quality of life
  • Lung diseases
  • Obstructive

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Exacerbations in subjects with alpha-1 antitrypsin deficiency receiving augmentation therapy. / Campos, Michael A; Alazemi, Saleh; Zhang, Guoyan; Wanner, Adam; Salathe, Matthias A; Baier, Horst; Sandhaus, Robert A.

In: Respiratory Medicine, Vol. 103, No. 10, 01.10.2009, p. 1532-1539.

Research output: Contribution to journalArticle

Campos, Michael A ; Alazemi, Saleh ; Zhang, Guoyan ; Wanner, Adam ; Salathe, Matthias A ; Baier, Horst ; Sandhaus, Robert A. / Exacerbations in subjects with alpha-1 antitrypsin deficiency receiving augmentation therapy. In: Respiratory Medicine. 2009 ; Vol. 103, No. 10. pp. 1532-1539.
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