Advocacy and Research

Scleroderma Scholarship I

By Riva Preil

June marks National Scleroderma Awareness Month. Scleroderma is a chronic autoimmune disease whose primary symptoms include fibrosis (hardening) of the skin and blood vessel changes. The underlying cause of scleroderma is unknown, however it tends to run in families, which indicates that there is a genetic component to the disease yet to be identified. Scleroderma affects the arterioles (small blood vessels) throughout the body, and it causes the endothelial and smooth muscle cells to die and be replaced by collagen. In addition, inflammatory cells (such as CD4 and helper T cells) enter the arteriole and interfere with normal arteriole functioning. This is why there is a strong correlation between scleroderma and Raynaud’s phenomenon, a disease associated with vasoconstriction of peripheral blood vessels which results in skin discoloration, feeling cold in the hands and feet, and possibly even skin atrophy.

The two main types of scleroderma are limited systemic scleroderma and diffuse systemic scleroderma. The limited version is less dangerous than the diffuse version, and there is a good prognosis and normal life expectancy. The areas mainly affected are the hands, arms, and face. Approximately one third of individuals with limited systemic scleroderma develop pulmonary arterial hypertension (elevated blood pressure), and it is therefore important for blood pressure to be monitored regularly. However, diffuse systemic scleroderma on the other hand, has a negative prognosis. It is a rapidly progressive degenerative disease which affects not only the skin, but also one (or more) internal organs such as the heart, kidneys, lungs, or esophagus. Five year survival after diagnosis of diffuse scleroderma is 70%, and ten year survival is 55%.

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