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Copyright ©ERS Journals Ltd 2007
1-Antitrypsin inhalation reduces airway inflammation in cystic fibrosis patients
1 Lung Research Group, Dr. von Hauner Children's Hospital, Ludwig Maximilians University, Munich, and 2 Bayer Healthcare - Biological Products, Q 30, Leverkusen, Germany.
CORRESPONDENCE: M. Griese, Dr. von Hauner Children's Hospital, Ludwig Maximilians University, Lindwurmstr 4, D-80337 Munich, Germany. Fax: 49 8951607872. E-mail: matthias.griese{at}med.uni-muenchen.de
Keywords: 
1-Antitrypsin, cystic fibrosis, elastase, interleukin-8, neutrophils
Received: April 5, 2006
Accepted October 10, 2006
The airways of cystic fibrosis (CF) patients are characterised by neutrophils that release high amounts of elastase overwhelming the local antiprotease shield. Inhalation of
In a prospective, randomised study, 52 CF patients received a daily deposition by inhalation of 25 mg AAT for 4 weeks targeting their peripheral or bronchial compartment. The levels of elastase activity, AAT, pro-inflammatory cytokines, neutrophils, immunoglobulin G fragments and the numbers of Pseudomonas aeruginosa were assessed in induced sputum before and after the inhalation period.
Inhalation of AAT increased AAT levels and decreased the levels of elastase activity, neutrophils, pro-inflammatory cytokines and the numbers of P. aeruginosa. However, it had no effect on lung function. No difference was found between the peripheral and bronchial inhalation mode.
In conclusion, although no effect on lung function was observed, the clear reduction of airway inflammation after
1-antitrypsin (AAT) may restore the protease–antiprotease balance and attenuate airway inflammation in CF airways. The aims of the present study were: 1) to assess the best deposition region for inhaled AAT by two different inhalation strategies; and 2) to examine the effect of 4 weeks of AAT inhalation on lung function, protease–antiprotease balance and airway inflammation in CF patients. 1-antitrypsin treatment may precede pulmonary structural changes. The 1-antitrypsin deposition region may play a minor role for 1-antitrypsin inhalation in cystic fibrosis patients.
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