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This information was reviewed and approved by Mehrnaz Maleki Fischbach, MD (9/1/2019).

Scleroderma (systemic sclerosis) is a rare disease. About 10-20 new cases per 1 million people are diagnosed each year. 

People of all races and ethnic backgrounds get scleroderma, but about 75 percent of people with the disease are women. It can occur at any age. However, scleroderma most commonly begins when people are between the ages of 30 and 60 years old.

While the cause of scleroderma is unknown, we do know that it is an autoimmune disease. This means the body's natural immune system does not behave normally. Instead of serving to fight off infections from bacteria, viruses and the like, the immune system of a person with scleroderma attacks its own body. This damages blood vessels.

The body's response to damage to its own blood vessels is to make specific proteins, called collagen. The collagen repairs the damage by forming diffuse scars (fibrosis) throughout the blood vessels. The excess collagen can deposit in the skin, in turn causing the many skin changes seen with scleroderma. In addition, the fibrosis (growth of scar tissue) of the blood vessels leads to involvement of many of the internal organs.

Twenty-five to sixty percent of people with scleroderma are often diagnosed with ILD. This number is higher when more of the skin is affected. Ten to fifteen percent of people with scleroderma may be diagnosed with pulmonary hypertension. This is an increase in the blood pressure in the lungs.

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