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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.