RT Journal Article
SR Electronic
T1 Multiple breath washout for monitoring alpha-1-antitrypsin deficiency related lung disease
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1487
VO 42
IS Suppl 57
A1 Susanne Fuchs
A1 Matthias Griese
A1 Doris Staab
A1 Klaus Pittschieler
A1 Frank Ahrens
A1 Ernst Rietschel
A1 Nikolaus Schwerk
A1 Wolfgang Gleiber
A1 Winfried Baden
A1 Thomas Köhnlein
A1 Robert Bals
A1 Monika Gappa
YR 2013
UL http://erj.ersjournals.com/content/42/Suppl_57/1487.abstract
AB Patients with alpha-1-antitrypsin deficiency (α1-AT deficiency) with a PiZZ genotype are at high risk to develop fatal emphysema during adulthood. Spirometry is commonly used for monitoring these patients. However early manifestation of emphysema is suspected within the peripheral airways that are not accessible by spirometry. We hypothesized that the Lung Clearance Index (LCI) from Multiple Breath Nitrogen Washout (MBWN2) tests is sensitive for detecting ventilation inhomogeneity (VI) within the peripheral airways and may thus be suitable to bridge this diagnostic gap.We present preliminary data of an ongoing multi centre study assessing the clinical value of measuring the LCI in patients with α1-AT deficiency.89 patients (4-73 years, PiZZ) and 40 controls (7-84years) performed 2-3 single MBWN2 (EasyOne Pro LABTM) and subsequent spirometry.Mean (SD) LCI in patients was 9.0 (2.9) and 6.5 (0.72) in controls. Mean difference (95% ci, p-value) between the groups was -2.49 (-3.14;-1.84, <0.001). Mean FEV1-z (SD) in patients was -1.36 (1.8). In 31/89 patients LCI and FEV1-z were abnormal, in 32/89 LCI and FEV1-z were normal, in 1/89 FEV1-z was abnormal while LCI was normal and in 25/89 LCI was abnormal while FEV1-z remained normal.LCI discriminated significantly between patients and controls. 44% of the patients with a normal FEV1-z had an abnormal LCI potentially indicating early manifestation of α1-AT deficiency related lung disease that is not detectable using spirometry alone. We conclude from these preliminary data that the LCI reflects presence and extent of α1-AT deficiency related lung disease and detects these changes earlier than spirometry.This project is supported by Grifols.