Vascular Disease in Progressive Systemic Sclerosis (Scleroderma)

Not only is the cause of progressive systemic sclerosis unknown, but there is no general agreement on the point of pathological impact. However, clinical, morphological, and functional observations provide a consistent body of evidence indicating widespread vascular abnormalities in the disease. Although characteristic arterial lesions have been recognized for many years, their frequency has generally been considered to be insufficient to account for the varied manifestations of progressive systemic sclerosis. Recent evidence suggests that the capillary bed may be a primary site of injury, and diffuse devascularization of multiple tissues may be a major consequence. On the basis of evidence reviewed, progressive systemic sclerosis should be classified as a vascular disease, and the primary site of injury is at the microvascular level.