Suppressive effect of sarpogrelate hydrochloride on respiratory failure and right ventricular failure with pulmonary hypertension in patients with systemic sclerosis.

We evaluated the effect of 12 months of 300-mg oral sarpogrelate hydrochloride (SH) once daily on the symptoms of Raynaud's phenomenon, respiratory failure and cardiac function in seven patients with systemic sclerosis. Arterial blood gases, pulmonary function, mean pulmonary arterial pressure, left ventricular ejection fraction (LVEF), right ventricular ejection fraction (RVEF), white blood cell count, C-reactive protein and the plasma concentrations of fibrinopeptide A, beta-thromboglobulin, platelet factor 4 and thrombomodulin were evaluated before and 2 and 12 months after SH administration. After 2 and 12 months of SH administration, a significant decrease was found in the frequency and duration of Raynaud's phenomenon, as well as the coldness, numbness and pain of Raynaud's phenomenon. Respiratory failure, as estimated by Hugh-Jones classification, was significantly decreased, whereas the percentage carbon monoxide diffusion capacity was significantly increased. The mean pulmonary arterial pressure decreased significantly, as did plasma fibrinopeptide A, beta-thromboglobulin and platelet factor 4. There was no significant change in LVEF after 2 or 12 months, but after 12 months of SH administration, RVEF increased significantly. In conclusion, use of SH may prevent Raynaud's phenomenon, respiratory failure and right ventricular failure in patients with systemic sclerosis.