PMID- 33824583
OWN - NLM
STAT- MEDLINE
DCOM- 20210625
LR  - 20220421
IS  - 1178-2005 (Electronic)
IS  - 1176-9106 (Print)
IS  - 1176-9106 (Linking)
VI  - 16
DP  - 2021
TI  - There is No Fast Track to Identify Fast Decliners in Alpha-1 Antitrypsin 
      Deficiency by Spirometry: A Longitudinal Study of Repeated Measurements.
PG  - 835-840
LID - 10.2147/COPD.S298585 [doi]
AB  - BACKGROUND: It is known that lung function decline in Alpha-1 Antitrypsin 
      Deficiency (AATD) varies. Those with a rapid decline are at highest risk of 
      poorer outcomes but may benefit most from targeted treatments including 
      augmentation therapy. Current evidence suggests rapid decliners can be identified 
      after 3 years of serial follow-up. It would be advantageous to identify these 
      patients over a shorter time period, especially in mild disease. METHODS: 
      Post-bronchodilator spirometry was performed every 6 months for a total of 18 
      months (4 measurements) by PiZZ AATD patients (ex- or never-smokers) either 
      without spirometric COPD or with mild COPD. Where possible, retrospective 
      spirometry data were included. Decline was assessed using 2 (baseline and 6 
      month) or four measurements (including baseline, 6, 12 and 18 months) and 
      compared to retrospective decline rates using annual measurements over 3 years. 
      RESULTS: Seventy-two PiZZ AATD patients were included, with 27 having at least 
      three years of retrospective, annual spirometry. 18-month progression obtained by 
      linear regression showed variable degrees of change with 29 showing no decline, 8 
      showing slow decline and 35 showing rapid decline. Bland-Altman plots showed that 
      there was no overall agreement between predicted rate of decline using data 
      obtained over 6 months and that obtained over 18 months. Furthermore, there was 
      no agreement between rate of decline from either 6 or 18 months' data when 
      compared to data collected over 3 years. The positive predictive value for rapid 
      decline with 18 months of data compared to 3 years was only 50.0%. CONCLUSION: 
      This study suggests serial lung function over 18 months cannot identify AATD 
      patients who have rapidly declining lung function. There is an urgent need for 
      different biomarkers to help identify these patients at the earliest opportunity.
CI  - (c) 2021 Stockley et al.
FAU - Stockley, James A
AU  - Stockley JA
AUID- ORCID: 0000-0002-4070-1923
AD  - Lung Function & Sleep Department, University Hospitals Birmingham NHS Foundation 
      Trust, Birmingham, B15 2GW, UK.
FAU - Stockley, Robert A
AU  - Stockley RA
AUID- ORCID: 0000-0003-3726-1207
AD  - Respiratory Department, University Hospitals Birmingham NHS Foundation Trust, 
      Birmingham, B15 2GW, UK.
FAU - Sapey, Elizabeth
AU  - Sapey E
AUID- ORCID: 0000-0003-3454-5482
AD  - PIONEER Health Data Hub in Acute Care, Institute of Inflammation and Ageing, 
      University of Birmingham, Birmingham, B15 2GW, UK.
LA  - eng
GR  - MR/L008335/1/MRC_/Medical Research Council/United Kingdom
PT  - Journal Article
DEP - 20210329
PL  - New Zealand
TA  - Int J Chron Obstruct Pulmon Dis
JT  - International journal of chronic obstructive pulmonary disease
JID - 101273481
RN  - 0 (alpha 1-Antitrypsin)
SB  - IM
MH  - Humans
MH  - Longitudinal Studies
MH  - *Pulmonary Disease, Chronic Obstructive/diagnosis
MH  - Retrospective Studies
MH  - Spirometry
MH  - alpha 1-Antitrypsin
MH  - *alpha 1-Antitrypsin Deficiency/diagnosis
PMC - PMC8018552
OTO - NOTNLM
OT  - alpha-1 antitrypsin deficiency
OT  - decline
OT  - lung function
OT  - obstructive airways disease
COIS- Professor Robert A Stockley is steering committee for Vertex, advisory board for 
      Z factor, and chair of Data Safety Monitoring Board for Kamada; he also reports 
      grants and/or personal fees from CSL Behring, Mereo biopharma, took part in the 
      laboratory analyses for Takeda, during the conduct of the study. Professor 
      Elizabeth Sapey reports grants from Alpha-1 Foundation, during the conduct of the 
      study; grants from HDR-UK, The Wellcome Trust, MRC, British Lung Foundation, 
      NIHR, and EPSRC, outside the submitted work. The authors report no other 
      conflicts of interest in this work.
EDAT- 2021/04/08 06:00
MHDA- 2021/06/29 06:00
PMCR- 2021/03/29
CRDT- 2021/04/07 06:22
PHST- 2020/12/22 00:00 [received]
PHST- 2021/03/14 00:00 [accepted]
PHST- 2021/04/07 06:22 [entrez]
PHST- 2021/04/08 06:00 [pubmed]
PHST- 2021/06/29 06:00 [medline]
PHST- 2021/03/29 00:00 [pmc-release]
AID - 298585 [pii]
AID - 10.2147/COPD.S298585 [doi]
PST - epublish
SO  - Int J Chron Obstruct Pulmon Dis. 2021 Mar 29;16:835-840. doi: 
      10.2147/COPD.S298585. eCollection 2021.