Ten year survival of a patient with advanced pulmonary hypertension and mixed connective tissue disease treated with immunosuppressive therapy.

Pulmonary hypertension is the most frequent cause of death in patients with mixed connective tissue disease. The pulmonary hypertension is progressive and difficult to treat effectively. These patients experience considerable morbidity secondary to diminished cardiac function. We describe a case in which sequential administration of cyclophosphamide and cyclosporine A favorably altered the clinical and hemodynamic course of this serious complication.