@article {SylvesterP2001, author = {Karl P. Sylvester and Jess Matthews and Matthew A. Rutter and Tom Dymond and Ravi Mahadeva}, title = {Lung function decline in patients with severe alpha1-antitrypsin deficiency}, volume = {44}, number = {Suppl 58}, elocation-id = {P2001}, year = {2014}, publisher = {European Respiratory Society}, abstract = {Severe alpha1-antittrypsin deficiency (AATD) is the only known genetic cause of emphysema. There appears to be variability in the clinical course of AATD associated COPD.We present preliminary characterisation of the change in FEV1 in a cohort of patients with severe AATD.Forced expiratory flow-volume loops measured to ATS/ERS guidelines were retrospectively reviewed from 38 consecutive patients. Values were expressed as mls.There were 38 individuals (18 males), 1 smoker/28 ex-smokers/9 never smokers. Median age (yrs); 64 (16-81) with at least 3 years follow-up. 52.6\% had a median decline in FEV1 of 66mls/year (35-218), greater than the mean rate of decline in COPD (Vestbo J et al, NEJM 2011), including 5 never smokers. 47.4\% of the cohort had a decline in FEV1 \< 33mls/yr or improvement in FEV1; median 23 (-27 -133), 4 never smokers. 50\% of these had an improvement in FEV1 with a median (range) 52 (21 to 133) mls/yr. 71\% of never smokers and smokers declined >=33mls per year.The rate of decline was lower in those with FEV1 \< 50\%.View this table:Table 1. FEV1 decline dependent on starting valueThere is considerable variability in change in FEV1 in patients with severe AATD even in never smokers. A substantial proportion of patients had a faster rate of decline than COPD patients. Interestingly there was also an improvement in lung function in some individuals following attendance at a specialist centre. Further analysis of other lung function parameters and environmental factors e.g. occupation is required which should aid counselling patients and targeting therapies.}, issn = {0903-1936}, URL = {https://erj.ersjournals.com/content/44/Suppl_58/P2001}, eprint = {https://erj.ersjournals.com/content}, journal = {European Respiratory Journal} }