Systemic Sclerosis (Scleroderma)

Systemic sclerosis, also known as scleroderma, is an autoimmune rheumatic disease that affects the skin and internal organs. Systemic sclerosis is characterized by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse.  

Causes

The exact cause of systemic sclerosis is unknown, but it is believed to be an autoimmune disorder, which means that the body’s immune system mistakenly attacks healthy tissue. Other factors that may contribute to the development of systemic sclerosis include genetics and environmental factors such as exposure to certain chemicals.

Symptoms

Some common symptoms include:

• Patches of hard or thickened skin
• Swelling of fingers and toes
• Ulcers occurring on fingertips
• Chalky hard swellings forming in the skin
• Dry or itchy skin with less hair growth

Other symptoms may include:

• Heartburn
• Trouble swallowing
• Shortness of breath
• Aches and pains in several joints
• Inflammation of the muscles (myositis), with muscle pain and weakness

Treatment

There is no cure for systemic sclerosis, but treatments can help manage symptoms and prevent complications. Treatment options may include medications to control symptoms such as high blood pressure, heartburn, and Raynaud’s phenomenon.

Other medications may be used to suppress the immune system and slow the progression of the disease.

Some common ways to manage the symptoms of systemic sclerosis include avoiding cold temperatures and stress, quitting smoking, and getting regular exercise. Other ways may include physical therapy and occupational therapy to help maintain mobility and function.

For more information on diagnosis and treatment of systemic sclerosis, please see the Scleroderma Clinic.