Small airways dysfunction and “early” COPD due to alpha-1 antitrypsin deficiency

2024

Closed access Metadata (CSL-JSON)
DOI 10.1183/13993003.congress-2024.pa3474
Data confidence 70 medium
How this score is calculated

It is built from checkable properties of the record itself: whether the article has a DOI, how many independent sources confirmed its data, whether the required fields are filled in, and how recent the latest confirmation is. Disagreement between sources and a missing identifier lower it. A value of 80 or above means the record was published without further checking; 60 to 79 means it is published at reduced confidence, with a manual check advisable. A record verified by an editor by hand is never pushed below 90 by a recalculation. This scores our data about the article, not the article itself.

Abstract

Background: There is increasing interest in small airways dysfunction (SAD) as the earliest physiological evidence of developing COPD. Previously we have shown that low maximal mid-expiratory flow (MMEF) in Alpha-1 Antitrypsin Deficiency (AATD) subjects is associated with a faster rate of FEV1 decline on average (Stockley J et al ERJ 2017;49:1602055). We sought to determine if other tests previously proposed as markers of SAD could show similar potential. Methods: Group 1 (n=33) had FEV1/FVC>70% and MMEF >80% pred (no SAD, no COPD) and Group 2 (n=24) had FEV1/FVC>70% and MMEF<80% predicted (SAD, no COPD). We compared area under the expiratory flow-volume curve (AEX), lung clearance index (LCI), oscillometry (R5-R20, X5, Fres), and total/effective specific resistance (SRtot, SReff) by plethysmography using an independent t-test or Mann Whitney-U test, depending on distribution. Results: AEX, two oscillometry parameters (X5 and Fres), and SRtot were different in Group 2 (Table 1). erj;64/suppl_68/PA3474/TB1 T1 TB1 Group 1 Group 2 p AEX %Pred 121.4 (4.1) 96.5 (5.2) <0.0001 LCI 7.70 (7.01, 8.96) 8.77 (7.88, 9.88) ns R5-R20 0.020 (0.001, 0.054) 0.029 (0.020, 0.049) ns XS -0.673 (0.005) -0.908 (0.007) <0.01 Fres 8.78 (7.76, 10.92) 11.26 (8.76, 12.81) 0.02 SReff %Pred 60.9 (52.6, 75.5) 68.5 (60.0, 81.6) ns SRtot %Pred 82.5 (3.8) 93.7 (4.9) 0.04 Table 1: Comparisons in AATD between Group 1 and Group 2. Data presented as Mean (SE) or Median (LQ,UQ). Conclusions: In AATD, there are other markers of SAD beyond MMEF that may identify patients with early disease and could potentially identify more rapid FEV1 decliners, although this will need future investigation. Most of these are non-volitional and may be more achievable than spirometry.