Augmentation therapy in emphysema patient with ZZ α-1-antitrypsin deficiency

Summaryα-1-antitrypsin deficiency (AATD) (PiZZ) emphysema patient was treated with 60–120 mg/kg body weight of Prolastin® weekly or every second week over 8 years (from 1992 until 2000). The therapy was re-started in summer 2002 and terminated in autumn 2004. Results from lung function tests, liver enzymes and plasma inflammatory markers are presented from years 2002 and 2004, and 7 months after termination of treatment. No changes in lung function tests, liver enzymes or plasma inflammatory markers were observed during a period of study. Blood neutrophils were isolated in October 2004, before and after Prolastin® infusion, and in October 2005. Neutrophils were analyzed for baseline and zymosan-induced interleukine-8 (IL-8) release. In response to zymosan neutrophils isolated after Prolastin® infusion released significantly lower levels of IL-8 than neutrophils obtained before Prolastin® infusion or after termination of treatment. Thus, blood neutrophils exhibit diminished capacity to release IL-8 after direct use of the augmentation therapy.