ABSTRACT
Scleroderma is a chronic autoimmune inflammatory disease with widespread fibrosis of the skin and internal organs. İt is characterized by vascular injury, immune activation, inflammation and fibrosis. Controversies on the pathogenesis of scleroderma still exist. Scleroderma is the most unsuccessfully treated disease among the rheumatic disorders. Prognosis is grave and mortality is due to cardiac, renal or pulmonary involvements. Despite prominent advances in the treatments of scleroderma related organ involvements like renal crisis, interstitial lung fibrosis and pulmonary arteriel hypertension, it has still mortal course.