HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) All Versions of this Article: 33/6/1338    most recent 09031936.00061208v1 Alert me when this article is cited Alert me if a correction is posted Permissions Request Permissions Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Web of Science (1) Citing Articles via Google Scholar Google Scholar Articles by Dawkins, P. A. Articles by Stockley, R. A. Search for Related Content PubMed PubMed Citation Articles by Dawkins, P. A. Articles by Stockley, R. A.

Rate of progression of lung function impairment in ₁-antitrypsin deficiency

Lung Investigation Unit, University Hospital Birmingham and University of Birmingham, Birmingham, UK.

CORRESPONDENCE: P. A. Dawkins, Dept of Respiratory Medicine, New Cross Hospital, Wednesfield Road, Wolverhampton, WV10 0QP, UK. Fax: 44 1902695725. E-mail: p.a.dawkins{at}bham.ac.uk

Keywords: ₁-antitrypsin deficiency, chronic obstructive pulmonary disease, disease progression, lung function tests

Received: April 21, 2008
Accepted December 29, 2008

The aim of the present study was to identify ₁-antitrypsin (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 (FEV₁) was 49.9 mL. The greatest FEV₁ decline occurred in the moderate severity group (FEV₁ 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 (FEV₁ <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 FEV₁ 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 FEV₁ decline was independently associated with BMI, BDR, exacerbation rate and high physical component 36-item short-form health survey scores.

In PiZ ₁-AT-deficient patients, FEV₁ decline was greatest in moderate disease, unlike K_CO decline, which was greatest in severe disease. The FEV₁ decline showed associations with BDR, BMI, sex and exacerbation rate.