The etiology of scleroderma remains unknown. However, it is believed that the vascular lesions found in involved organs have a pathogenesis related to endothelial cell injury (1).
Pulmonary hypertension is the most common cause of acute dyspnea in patients with systemic sclerosis and is thought to indicate pulmonary vascular involvement with proliferative lesions of scleroderma. Therapy with glucocorticoids has been effective in some patients, when pulmonary hypertension occurs without pulmonary interstitial fibrosis. When the two coexist, however, the pulmonary hypertension has usually been refractory to treatment (2).
Calcium entry blocking drugs are potent vasodilators which have been used to treat Raynaud's phenomenon (3,4) and primary pulmonary hypertension (5) with some success. Recently, in a patient with systemic sclerosis and pulmonary hypertension, we documented the efficacy of one of these drugsnifedipine.
Case report, VC, a 62-year-old man with a history of systemic hypertension and diabetes mellitus, was first admitted to the Bronx Municipal Hospital Center, Albert Einstein College of Medicine, in From the Albert Einstein College of Medicine, Department of Medicine, Divisions of Rheumatology and Cardiology, and Department of Radiology.