Systemic sclerosis

Systemic sclerosis. Systemic sclerosis is more frequent among women (sex ratio from 3/1 to 8/1) and its prevalence varies from 30 to 240 per millions. Two types of SSc are described depending on the extent of skin involvement : limited and diffuse SSc. Clinical manifestations include fibrosing lesions (skin and pulmonary fibrosis) and vascular abnormalities (Raynaud's phenomenon and pulmonary arterial hypertension (PAH)). Auto-antibodies have been identified in SSc patients, directed at centromere proteins, topoisomerase 1 or RNA polymerase III. SSc is associated with a poor outcome particularly in diffuse forms associated with pulmonary involvement (pulmonary fibrosis and/or PAH). SSc treatment associate symptomatic treatments as well as treatment of visceral complications (PAH, renal crisis) and immunosuppressive therapy in patients with recent onset diffuse SSc and/or rapidely progressing interstitial lung disease.