Clinical problems - The lungs

Among patients with systemic sclerosis (SSc, scleroderma), pulmonary disease has become the number one cause of death. Pulmonary involvement, in the absence of renal or cardiac involvement, is a major contribution to morbidity (median survival 78+/-17 months). In this article, the two major pulmonary manifestations of SSC-pulmonary hypertension and interstitial lung disease-are described in terms of clinical features, current concepts of pathogenesis, and treatment options.