The treatment of advanced pulmonary emphysema is a serious and depressing problem for respiratory physicians. The patients, mainly smokers or former smokers, suffer from progressive disability and have a reduced life expectancy. Such patients are short of breath on mild exertion and have hyperresonant chests and quiet or absent breath sounds. Destruction of the alveolar walls is a key feature of the disease.1

Emphysema can be divided into pathological types according to the distribution of the enlarged air spaces within the acinus (the section of the lung that is supplied by a single terminal bronchiole2). Two types seem important. In the centriacinar form the enlarged air spaces are in the centre of the acinus close to the terminal bronchioles, and these lesions are usually situated in the upper zones of the lungs. In the panacinar form the whole acinus is affected and lesions may be distributed throughout the lung or concentrated in the lower zones.

The pathogenesis of emphysema remained obscure until Swedish workers discovered the association between panacinar emphysema and a hereditary deficiency of (alpha)₁-proteinase inhibitor.3 …