Therapeutics: Alpha-1 antitrypsin augmentation therapy

Michael A Campos, Jorge Lascano

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations

Abstract

Subjects with alpha-1 antitrypsin deficiency who develop pulmonary disease are managed following general treatment guidelines, including disease management interventions. In addition, administration of intravenous infusions of alpha-1 proteinase inhibitor (augmentation therapy) at regular schedules is a specific therapy for individuals with AATD with pulmonary involvement. This chapter summarizes the manufacturing differences of commercially available formulations and the available evidence of the effects of augmentation therapy. Biologically, there is clear evidence of in vivo local antiprotease effects in the lung and systemic immunomodulatory effects. Clinically, there is cumulative evidence of slowing lung function decline and emphysema progression. The optimal dose of augmentation therapy is being revised as well as more individualized assessment of who needs this therapy.

Original languageEnglish (US)
Title of host publicationMethods in Molecular Biology
PublisherHumana Press Inc.
Pages249-262
Number of pages14
Volume1639
DOIs
StatePublished - 2017

Publication series

NameMethods in Molecular Biology
Volume1639
ISSN (Print)1064-3745

Keywords

  • Alpha-1 antitrypsin deficiency
  • Alpha-1 proteinase inhibitor
  • Augmentation therapy
  • Clinical trials
  • COPD
  • Emphysema

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics

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  • Cite this

    Campos, M. A., & Lascano, J. (2017). Therapeutics: Alpha-1 antitrypsin augmentation therapy. In Methods in Molecular Biology (Vol. 1639, pp. 249-262). (Methods in Molecular Biology; Vol. 1639). Humana Press Inc.. https://doi.org/10.1007/978-1-4939-7163-3_25