Diffuse (interstitial) lung disease comprises a wide variety of condit
ions, individually relatively uncommon but collectively being found in
approximately 50 per 100 000 population. Some of these diseases are o
f known aetiology but others are not. It has been suggested that the e
nvironment is a major contributory factor in this group of diseases. H
owever, since not all individuals exposed to a common environment deve
lop interstitial diseases, it can be hypothesised that there is a gene
tic predisposition to their development. These diseases cause major mo
rbidity and mortality due to lung injury and fibrosis. It follows that
, if individuals who are genetically predisposed to develop diseases c
haracterised by lung injury and fibrosis can be identified, then manag
ement strategies can be designed which will attempt to identify and tr
eat early disease and, in the longer term, to develop targeted genetic
interventional approaches to treatment.