Systemic sclerosis is a rare, systemic autoimmune disease, characterized by inflammation, vasculopathy and fibrosis of the skin and internal organs. The disease is associated with a significantly increased morbidity and mortality, and can be rapidly progressive. Interstitial lung disease, renal hypertensive crisis, cardiac involvement and pulmonary arterial hypertension are life-threatening complications. Early treatment with immunosuppressive drugs can prevent progression and decrease morbidity and mortality.