National Centre for Pharmacoeconomics

National Centre for

NCPE Ireland

Human alpha1-proteinase inhibitor (Respreeza®)

Respreeza® is indicated for maintenance treatment, to slow the progression of emphysema in adults with documented severe alpha1-proteinase inhibitor deficiency (e.g. genotypes PiZZ, PiZ(null), Pi(null, null), PiSZ). Patients are to be under optimal pharmacologic and nonpharmacological treatment and show evidence of progressive lung disease (e.g. lower forced expiratory volume per second (FEV1) predicted, impaired walking capacity or increased number of exacerbations) as evaluated by a healthcare professional experienced in the treatment of alpha1-proteinase inhibitor deficiency.


Rapid Review

Commenced Completed Outcome
20/01/2016 05/02/2016 Full Pharmacoeconomic Evaluation Recommended


Pharmacoeconomic Evaluation

Commenced Completed Outcome
13/06/2016  09/12/2016 Reimbursement Not Recommended

The manufacturer CSL Behring confirmed that the compassionate access programme is being extended until the end of January 2017 when the programme is due to end.


The cost-effectiveness of human alpha-1 proteinase inhibitor (Respreeza®) has not been demonstrated and therefore is not recommended for reimbursement.