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Systemic sclerosis


Authors: M. Tomčík
Authors‘ workplace: Revmatologický ústav a Revmatologická klinika 1. LF UK, Praha
Published in: Kardiol Rev Int Med 2014, 16(5): 414-419
Category: Internal Medicine

Overview

Systemic sclerosis (SSc) is a rare, chronic connective tissue disease affecting the skin, musculoskeletal system and internal organs. SSc occurs predominantly in women, begins usually in middle age, and the overall survival rate is reduced –  70% of patients survive 10 years. Despite the advances in pharmacotherapy of organ complications and new insights into the pathogenesis of SSc, there is no effective treatment for this serious disorder. The aim of this review article is to introduce this rare disease, its main symptoms, basic principles of dia­gnosis and current treatment, and contribute to early dia­gnosis and better prognosis of patients with SSc.

Keywords:
systemic sclerosis –  rheumatic diseases –  connective tissue disease –  fibrosis –  vasculopathy –  autoimmunity


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