Scleroderma

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Overview

Scleroderma, also called systemic sclerosis (SSc), is a chronic autoimmune disease that can affect a number of body systems (i.e., a multisystemic disorder). In patients with systemic sclerosis, certain cells in the body overproduce collagen, which is a protein found in connective tissue (e.g., skin, bone, cartilage, ligaments). Excess collagen is deposited throughout the body, causing hardening of the skin and tissues (called fibrosis), damaging blood vessels, and affecting internal organs (e.g., gastrointestinal tract, heart, lungs, kidneys, nervous system).

Scleroderma is both a rheumatic disease (i.e., disease that causes inflammation and pain in the muscles, joints, and fibrous tissue) and a connective tissue disease (i.e., disease that affects the skin, bones, and tendons). It is a rare disorder that results from an immune system response.

The immune system is a complex network of organs, cells (e.g., leukocytes, lymphocytes, phagocytes, B cells, CD8+ cells), and proteins that protects the body from disease and illness. It attacks foreign organisms (e.g., germs), identifies and destroys abnormal cells, and flushes dead and damaged cells out of the body. In autoimmune diseases such as scleroderma, the immune system attacks normal cells in the body, causing damage and inflammation.

Excessive collagen production, blood vessel damage, and the formation of abnormal antibodies (autoantibodies) all play a role in the development of scleroderma.

Incidence and Prevalence
According to the Arthritis Foundation, systemic sclerosis affects between 100,000 and 165,000 people in the United States. The disease is more common in adults between the ages of 30 and 50, and is 3 to 5 times more common in women than men. Scleroderma also may affect children and the elderly.

The diffuse (widespread) form of the disease occurs more often in African American women than in Caucasian women. The highest prevalence of systemic sclerosis occurs in Choctaw Native Americans in Oklahoma.

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