Management of interstitial lung disease in scleroderma

Interstitial lung disease (ILD) is now the major cause of mortality in patients with systemic sclerosis (scleroderma) after dramatic reduction in death caused by scleroderma renal crisis, because of the regular use of angiotensin-converting enzyme inhibitors. Treatment of ILD in scleroderma has been somewhat haphazard and treatment protocol often been borrowed from the experience gained from treating ILD of unknown aetiology. The purpose of this review is to outline recent advances and current concepts regarding the management of these ILDs.