Scleroderma is a chronic disease characterized by persistent inflammation, immune system attack on body tissues, blood vessel abnormalities and tissue scarring. Although scleroderma is a systemic (body-wide) disease, it manifests differently from one person to another. Some people exhibit widespread skin thickening, while others have few skin changes. The lungs, musculoskeletal system, gastrointestinal tract, kidneys and heart can also be variably involved in scleroderma. Medications used for scleroderma can reduce the immune system's mistaken attack on body tissues and relieve organ-specific symptoms of the disease. The National Institute of Arthritis and Musculoskeletal and Skin Diseases estimates between 40,000 and 165,000 people in the United States have scleroderma.
Anti-Inflammatory Medications
Inflammation is an immune system response that normally functions to help clear infections and heal wounded tissue. In scleroderma, inflammation occurs without cause leading to tissue damage. Scleroderma can cause painful inflammation in the joints and muscles. The inflammation may also involve the protective tissues that cover the heart and lungs. Anti-inflammatory medications, including nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, are frequently used to control scleroderma-related inflammation. The dose and duration of treatment with these drugs depends on individual response. Some people with severe scleroderma need to stay on an anti-inflammatory medication long-term.
Immunosuppressant Medications
A primary function of the immune system is to protect the body from invasion by infectious agents. It must distinguish what is "self" versus what is "not self" in order to correctly direct its activities. With scleroderma, the immune system mistakes the body's own tissues for invaders and attacks them causing damage. Immunosuppressant medications reduce the activity of the immune system, thereby decreasing the damage caused by its errant attack on body tissues. Cyclophosphamide, methotrexate, antithymocyte globulin, cyclosporine and mycophenolate mofetil are immunosuppressant medications that may slow scleroderma disease progression.
In a 2007 study published in the "American Journal of Respiratory and Critical Care Medicine," lead author Dr. Donald Tashkin reported 12 months of cyclophosphamide treatment led to improved lung function and overall health status among people with scleroderma. However, the research team also noted these improvements were lost within two years after the cyclophosphamide treatment ended. Researchers continue to actively investigate medication regimens that may halt scleroderma disease progression long-term.
Vasodilators
People with scleroderma have widespread vasospasms--blood vessel constriction that reduces the delivery of oxygen and nutrients to the tissues. To control these vasospasms, doctors prescribe drugs called vasodilators, which cause the blood vessels to relax restoring normal blood flow. The calcium channel blocker nifedipine can reduce vasospasms in the heart and skin. Other vasodilating drugs, including benazepril, captopril, enalapril and lisinopril, are used to reduce vasospasms affecting the kidneys. Prostacyclin and bosentan counteract scleroderma-related vasospasms in the lungs. The choice of what vasodilator to use and when is dictated by the individual's changing manifestations of the disease.
