Les défaillances viscérales graves de la sclérodermie systémique

Systemic sclerosis (SSc) is a rare auto-immune disease involving immune cells, fibroblasts and endothelial cells. There are 2 main forms depending of the cutaneous extend, limited and diffuse forms. Whatever the form, all are associated with at high risk of visceral involvement particularly digestive, pulmonary, heart and renal which lead to the increase risk of mortality. Scleroderma renal crisis represents the classic manifestation of kidney involvement in SSc with newly severe hypertension, microangiopathy haemolytic anemia and renal failure. It occurs particularly in early, rapidly progressive, diffuse forms. Pulmonary hypertension is a rapid live threatening complication that occurs in about 10% of the patients. Dyspnea on exertion is usually the first and deceptive manifestation. World Health Organization guidelines propose to detect yearly pulmonary hypertension in SSc patients using echocardiography, diagnostic confirmation needs right heart catheterization.. Heart involvement is a frequent but mostly underdiagnosed complication. It can be the consequence of myocardial fibrosis or microangiopathy or secondarily to either lung or kidney involvement. The involvement of the lung interstitium in SSc is characterized by early alveolitis with a slowly or rapidly evolution to pulmonary fibrosis. The risk of a rapid evolution is particularly high in early diffuse SSc. Cyclophosphamide must be discussed in evolutive cases. Gastro-intestinal tract involvement results indigestive dysmobility, possible from the esophagus to the rectum resulting in heartburn, dysphagia, diarrhea, constipation, intestinal distension, malnutrition and sometimes fecal incontinence. In severe malabsorption enteral nutrition is needed. A regular visceral check-up, the adequate use of calcium channel blockers, converting inhibitor enzyme, proton pump inhibitors, the development the last 15 years of new drugs like prostacyclins and analogs, inhibitors of endothelin receptor explain an improvement in survival.