Sclérodermie systémique

Abstract

Systemic sclerosis is a rare auto-immune disease that involves immune cells, fibroblasts and endothelial cells. Depending on the cutaneous extend, the sclerosis may be either limited or diffuse. Whatever the form, all are associated with a high risk of visceral involvement, particularly digestive, pulmonary, cardiac and renal, resulting in an increased risk of mortality. Sclerodactily and digital ulcers alter considerably the patients' quality of live. Regular visceral check-up, adequate use of calcium channel blockers, ACE inhibitors and proton pump inhibitors, and the development these past 15 years of new drugs such as prostacyclins and analogues, endothelin receptor inhibitors, improve the patients survival.