Abstract
Systemic sclerosis (SSc) is a rare connective tissue disease characterized by fibrosis of the skin and internal organs and a vasculopathy affecting the micro- and macro-vasculature. The pathogenesis of SSc involves the interplay between vascular injury and dysregulation of the immune response with resultant fibrosis of various target organs. More than 90% of patients with SSc suffer from Raynaud’s phenomenon, a reversible vasospastic disorder induced by cold or stress, which results in typical white, blue, and red color changes of the distal extremities from decreased perfusion (Block JA, Sequeira W, 2001. Raynaud’s phenomenon. Lancet 357(9273), 2042–8). Other clinical features that are common in patients with SSc in addition to cutaneous sclerosis include pulmonary disease (interstitial lung disease and pulmonary hypertension), gastrointestinal dysmotility and malabsorption, digital ulcerations, inflammatory myositis and arthritis, cardiac and renal disease.
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