PT  - JOURNAL ARTICLE
AU  - Susanne Fuchs
AU  - Matthias Griese
AU  - Doris Staab
AU  - Klaus Pittschieler
AU  - Frank Ahrens
AU  - Ernst Rietschel
AU  - Nicolaus Schwerk
AU  - Wolfgang Gleiber
AU  - Winfried Baden
AU  - Thomas Köhnlein
AU  - Robert Bals
AU  - Monika Gappa
TI  - Lung clearance index in alpha-1-antitrypsin deficiency - A follow up
AID  - 10.1183/13993003.congress-2016.PA4130
DP  - 2016 Sep 01
TA  - European Respiratory Journal
PG  - PA4130
VI  - 48
IP  - suppl 60
4099  - http://erj.ersjournals.com/content/48/suppl_60/PA4130.short
4100  - http://erj.ersjournals.com/content/48/suppl_60/PA4130.full
SO  - Eur Respir J2016 Sep 01; 48
AB  - We have shown that the Lung Clearance Index (LCI) derived from Multiple Breath Nitrogen Washout (MBWN2) is more sensitive for detecting pulmonary changes than spirometry, with an abnormal LCI in almost 40% of patients with a normal FEV1. The aim of this follow up study was to assess the natural course of lung function in A1ATD using MBWN2 and spirometry.117 patients with a normal FEV1 at baseline were identified from our A1ATD cohort. MBWN2 and comparative spirometry using the EasyOne Pro LABTM (ndd Switzerland) were performed according to international standards.66/117 (6,7-72,7 years) eligible subjects participated. Mean (SD) interval between the two tests was 1,7 (0,4) years. At baseline and follow up, mean (SD) LCI was 7,3 (1,18) and 7,3 (1,46) respectively (95% ci -0,161; 0,228, p= 0,730 ); mean FEV1-z (SD) was -0,20 (0,73) and -0,28 (0,97) (95% ci -0,062;0,222, p= 0,265) with no significant change between the test occasions in patients with either normal or abnormal LCI at baseline. N=7 patients changed from a normal LCI at baseline to an LCI above the upper limit of normal, whereas n=5 other patients changed from normal FEV1-z at baseline to FEV1-z below the lower limit of normal.Longitudinal measurement of LCI in A1ATD is feasible. The LCI appears to be a robust parameter for assessing A1ATD related pulmonary changes. Our data suggest that even when early changes are detected by MBWN2, progress is slow so that there may be time for effective prevention and/or therapeutic intervention. Further follow up is necessary to understand early lung disease in A1ATD in relation to different lung function parameters.This project is supported by Deutsche Lungenstiftung and Grifols.