Human α1-proteinase inhibitor (Respreeza®) in α1-antitrypsin deficiency emphysema: a guide to its use in the EU

  title={Human $\alpha$1-proteinase inhibitor (Respreeza{\textregistered}) in $\alpha$1-antitrypsin deficiency emphysema: a guide to its use in the EU},
  author={Kate McKeage and Katherine A. Lyseng-Williamson},
  journal={Drugs \& Therapy Perspectives},
Augmentation with intravenous infusions of human α1-proteinase inhibitor [Respreeza® (EU); Zemaira® (USA)] slows the progression of emphysema in patients with severe α1-antitrypsin (AAT)-deficiency emphysema. In the randomized, double-blind RAPID trial in patients with severe AAT-deficiency emphysema, Respreeza®/Zemaira® significantly reduced the mean annual rate of lung density loss based on computed tomography imaging at total lung capacity (TLC), but not at functional residual capacity (FRC… Expand
1 Citations
Human α1-proteinase inhibitor (Respreeza®) in α1-antitrypsin deficiency emphysema: a profile of its use in the EU
The continued efficacy of Respreeza in slowing the progression of emphysema was demonstrated in a 2-year open-label extension of the RAPID trial, and lung density loss prior to treatment was not recovered. Expand


Alpha-1 proteinase inhibitors for the treatment of alpha-1 antitrypsin deficiency: safety, tolerability, and patient outcomes
The safety and tolerability profiles of AAT replacement in the context of patient outcomes and cost-effectiveness are addressed and future directions for work in this field are outlined. Expand
Augmentation Therapy for α1-Antitrypsin Deficiency
Although the lack of a large randomised controlled trial to date has precluded the definitive demonstration of clinical efficacy of intravenous AAT augmentation therapy, substantial evidence supporting its use in AAT-deficient individuals with moderate airflow obstruction has accumulated. Expand
Population pharmacokinetics of A1-PI in patients with Alpha-1 antitrypsin deficiency
Background: Alpha 1 proteinase inhibitor (A 1 -PI; Zemaira, CSL Behring) is used for augmentation therapy in patients with A 1 -PI deficiency (AATD). RAPID, a randomized, double-blind,Expand
Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial
BACKGROUND The efficacy of α1 proteinase inhibitor (A1PI) augmentation treatment for α1 antitrypsin deficiency has not been substantiated by a randomised, placebo-controlled trial. CT-measured lungExpand
Augmentation therapy for emphysema due to alpha-1-antitrypsin deficiency
Therapeutic regimens administered every two weeks appear to be safe and result in adequate trough serum concentrations, but less-frequent administrations result in trough levels below the target. Expand
Augmentation Therapy for α1 Antitrypsin Deficiency: A Meta-Analysis
This meta-analysis supports the conclusion that augmentation can slow lung function decline in patients with AAT deficiency and patients with moderate obstruction are most likely to benefit. Expand
Multi-Center Study: The Biochemical Efficacy, Safety and Tolerability of a New α1-Proteinase Inhibitor, Zemaira
The results demonstrate that the new plasma derived α1-PI (Zemaira) is bioequivalent to the currently available product Prolastin, is well tolerated, and safe with respect to the risk of viral transmission. Expand
α1-antitrypsin deficiency: what has it ever done for us?
The first five cases of α1-antitrypsin deficiency were originally published in 1963 and had a significant 50-year impact on many aspects of protein biochemistry, genetics, cell biology, and disease concepts outside the lung as well as the study of COPD in general. Expand
A pilot study comparing the purity, functionality and isoform composition of alpha-1-proteinase inhibitor (human) products
Zemaira demonstrated greater purity compared with Aralast and Prolastin, and Isoform ratios appeared to be altered in ArAlast, and warrant further investigation. Expand
Health-Related Quality of Life in Patients with Alpha-1 Antitrypsin Deficiency: The French Experience
The French registry provides important insights into the clinical characteristics of French patients with AATD-related emphysema, and a model including age, chronic bronchitis, dyspnea, diffusing lung capacity and 6-min walking distance explained 57% of the variation in the score. Expand