title: Rate of progression of lung function impairment in alpha1-antitrypsin deficiency.
abstract: The aim of the present study was to identify alpha(1)-antitrypsin (alpha(1)-AT)-deficient patients who had rapidly progressive disease. PiZ patients (n = 101) underwent annual lung function measurements over a 3-yr period, and the results were related to factors that may influence decline. The mean annual decline in forced expiratory volume in 1 s (FEV(1)) was 49.9 mL. The greatest FEV(1) decline occurred in the moderate severity group (FEV(1) 50-80% of the predicted value), with a mean annual decline of 90.1 mL, compared with 8.1 mL in the very severe group (FEV(1) <30% pred). However, annual decline in transfer coefficient of the lung for carbon monoxide (K(CO)) was greatest in the severe and very severe groups. When the whole group was divided into tertiles of FEV(1) decline, the fast tertile compared with the slow tertile had more patients with bronchodilator reversibility (BDR) (73 versus 41%; p = 0.010), more males (79 versus 56%; p = 0.048) and lower body mass index (BMI) (24.0 versus 26.1; p = 0.042). Logistic regression analyses confirmed that FEV(1) decline was independently associated with BMI, BDR, exacerbation rate and high physical component 36-item short-form health survey scores. In PiZ alpha(1)-AT-deficient patients, FEV(1) decline was greatest in moderate disease, unlike K(CO) decline, which was greatest in severe disease. The FEV(1) decline showed associations with BDR, BMI, sex and exacerbation rate.
authors.affiliation: Lung Investigation Unit, UniversityHospital Birmingham and University of Birmingham, Birmingham, UK. p.a.dawkins@bham.ac.uk
authors.forename: P A
authors.initials: PA
authors.lastname: Dawkins
authors.forename: C L
authors.initials: CL
authors.lastname: Dawkins
authors.forename: A M
authors.initials: AM
authors.lastname: Wood
authors.forename: P G
authors.initials: PG
authors.lastname: Nightingale
authors.forename: J A
authors.initials: JA
authors.lastname: Stockley
authors.forename: R A
authors.initials: RA
authors.lastname: Stockley
mesh_terms: D001993:Bronchodilator Agents / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D029424:Pulmonary Disease, Chronic Obstructive / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology*; D011656:Pulmonary Emphysema / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology*; D012042:Registries; D012044:Regression Analysis; D012129:Respiratory Function Tests; D012307:Risk Factors; D012720:Severity of Illness Index; D012907:Smoking; D018709:Statistics, Nonparametric; D011795:Surveys and Questionnaires; D014057:Tomography, X-Ray Computed; D019896:alpha 1-Antitrypsin Deficiency / Q000503:physiopathology* / Q000628:therapy
chemical_list: D001993:Bronchodilator Agents