Scleroderma

Systemic sclerosis, also known as scleroderma, is a rare autoimmune disease characterized by thickening and scarring of connective tissue throughout the body. The disease is estimated to affect about 100,000 people in the U.S. and 2.5 million worldwide. Fibrosis, the hallmark of the disease, can affect the skin and internal organs, including the lungs. Interstitial lung disease (ILD), one of the most frequent disease manifestations, can be debilitating and may become life-threatening. Approximately 25 percent of patients develop significant lung involvement within three years of diagnosis. ILD is the leading cause of death among people with systemic sclerosis.