This paper presents progress in management of systemic sclerosis, with respect of EULAR recommendation. In the first part we present efficacy of overall immunosupresive treatments and preliminary data of autologous stem cell transplantation based on randomized clinical trials. The second part concerns a treatment specific for target organs: Raynaud's phenomenon, alimentary tract involvement, interstitial lung disease, pulmonary arterial hypertension and scleroderma renal crisis. Despite intensive investigations disease modifying drug therapy is still unknown, but specific organ treatment increase survival and improve health related quality of life.