Skin Manifestations and Musculoskeletal Disease in SSc

Clinically, systemic sclerosis is dominated by two features: small vessel vasculopathy and organ fibrosis. These two pathological features are responsible for the clinical manifestations of Raynaud’s phenomenon, digital ulcers, renal crisis, pulmonary arterial hypertension, skin thickening, interstitial lung disease, cardiac complications, and other organ involvement. In spite of this commonality, there is a great deal of heterogeneity in disease expression in scleroderma with some individuals having mild involvement and others with severe and progressive disease.