Systemic Sclerosis

Systemic sclerosis is a heterogeneous rheumatic disease within the scleroderma spectrum of disorders. Although the terms ‘scleroderma’ and ‘systemic sclerosis’ are often used synonymously, a distinction is appropriate, with the latter used to describe conditions involving fibrosis of the skin and internal organs with microvasculopathy. Systemic sclerosis is relatively rare but important because of its high mortality and similarities to more common forms of organ-based fibrosis. These diverse conditions share several clinical features (particularly thickening of the skin resulting from dermal fibrosis and a high incidence of episodic peripheral vasospasm–Raynaud's phenomenon) and it is likely that common pathogenic processes underlie them.