Respreeza is indicated for maintenance treatment, to slow the progression of emphysema in adults with documented severe alpha 1 -proteinase inhibitor deficiency ( e.g. genotypes PiZZ, PiZ(null), Pi(null,null), PiSZ). Patients are to be under optimal pharmacologic and non-pharmacologic treatment and show evidence of progressive lung disease ( e.g . lower forced expiratory volume per second (FEV 1 ) predicted, impaired walking capacity or increased number of exacerbations) as evaluated by a healthcare professional experienced in the treatment of alpha 1 -proteinase inhibitor deficiency.