Systemic sclerosis

Abstract

Systemic sclerosis is a chronic multisystemic progressive disease that affects the skin and internal organs. Generally, Raynaud’s phenomenon and vascular changes are the first clinical manifestations followed by sclerotic and fibrotic changes. It is a rare disease: the incidence is 8-12 patients per million population and about 75 % of patients are woman. It has the highest case-specific mortality of any of the autoimmune rheumatic diseases: the 5-year survival is about 50-70 % when pulmonary hypertension is present. It is determined by the involvement of the heart and lung (pulmonary fibrosis, pneumonia) and in some populations of higher frequency of the kidney (uraemia, malignant hypertension).