The Microvascular Pathogenesis of Scleroderma: An Hypothesis

Excerpt

Systemic sclerosis (scleroderma) has lacked a therapeutically useful concept of pathogenesis. Speculation about immunologic mechanisms, neurogenic factors, vascular lesions, and collagen overproduction fails to explain the dominant clinical features. How does abnormal collagen, for example, explain tissue atrophy? How do autoantibodies cause Raynaud's phenomenon? How does arteriolar sclerosis cause capillary dilatation and edema? Unsurprisingly, therapeutic efforts initiated without a coherent concept of the disease have failed.

A pathogenetic concept of scleroderma must explain [1] antecedent Raynaud's phenomenon; [2] a sequence of edema, atrophy, and fibrosis; [3] arteriolar intimal fibrosis; [4] telangiectasia, capillary dilatation, and capillary hemorrhages; [5] increased prevalence of