Article Sections

Introduction

Scleroderma is a family of autoimmune connective tissue disorders characterized by an obliterative vasculopathy leading to fibrosis of the skin and internal organs.  It carries high morbidity and mortality due to involvement of the lungs, kidneys, heart, and gastrointestinal tract.

Pathogenesis

Scleroderma is thought to arise from the interplay of vasculopathy, autoimmunity, and fibrogenesis ( Figure 1).

Vasculopathy

The initial events in scleroderma are thought to begin in the small vessels.  Microvascular injuries may be related to antiendothelial antibodies, upregulated cell adhesion molecules (eg, ICAM-1), and altered balance of vasoconstrictors versus vasodilators (eg, high endothelin, low nitric oxide).  These changes may contribute to patients having increased vasospasticity of digital arteries (eg, Raynaud phenomenon) and pulmonary arteries.  Vessel lumens are narrowed due to neointimal hyperplasia, smooth muscle hypertrophy, and concentric "onion skin" fibrosis.

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Figures

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