Medical Aspects of Disease
Since the introduction of angiotensin-converting enzyme inhibitors and their remarkable success in managing scleroderma renal crisis, pulmonary complications have assumed ever greater importance as a source of morbidity and mortality in this disease. Prior to the use of captopril, renal failure was the leading cause of death in scleroderma. Now renal failure accounts for fewer than 10% of scleroderma-related deaths.
Explore this issueJanuary 2007
Also by this Author
At present, the majority (nearly 80%) of deaths now are attributable to interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Other pulmonary complications may occur, but ILD and PAH are the major complications experienced by our patients. (See Table 1, page 17.)
As pulmonary manifestations have assumed greater importance, new treatments now offer hope to SSc patients. Now that treatment is available, it is essential for the rheumatologist to screen patients for the presence of ILD and PAH, so that treatment can be instituted before the disease becomes irreversible. Similar to our current approach to patients with rheumatoid arthritis or lupus nephritis, there may be a window of opportunity to successfully intervene and treat ILD and PAH.