A recent follow-up study found that patients with pulmonary involvement (with no renal or cardiac involvement) survived a median of 78 +/- 17 months. Another study found death from SSc was most frequently due to pulmonary hypertension. Improved diagnostic modalities and better understanding of the pathophysiology of SSc lung disease are essential, because mortality from this SSc lung disease remains high. During the past decade, advances have been made in the understanding of the alveolitis of SSc lung disease. Although the inciting injury remains uncertain, a cascade of inflammatory and fibrosing mediators culminates in a chronic state of interstitial lung disease. There is increasing evidence that the fibrogenic cytokines PDGF and TGF-beta are major contributors to the pathophysiology of interstitial lung disease, including that of SSc. Future research aimed at modifying the biologic response to such cytokines may yield novel therapeutic approaches to the management of this type of lung disease. Similarly, improved understanding of mediators of vascular tone, such as endothelin and nitric oxide, may yield much-needed treatments for pulmonary hypertension.