Scleroderma

Scleroderma is a rare, chronic autoimmune disease that affects skin and internal organs. Scleroderma results from an overactive immune system causing inflammation and tissues changes, especially fibrosis. It often leads to skin tightening and thickening, and can affect joints, muscles, heart, lungs, kidneys, blood vessels or intestines. It affects mostly women between the ages of 30 and 50. Children may get a juvenile form of scleroderma. While there is no cure for scleroderma, there are treatments to help manage symptoms and improve quality of life.

What Are the Signs/Symptoms?

Scleroderma symptoms may range from minor to life-threatening. An early, common sign of scleroderma is color changes to the fingers called Raynaud’s phenomenon. Fingers or toes may look red, white, or blue, especially in cold temperatures and may be swollen, numb, painful or develop ulcers. In addition to Raynaud’s phenomenon, patients can have other symptoms depending on the involvement of joints, muscles, and internal organs including the heart, lungs, blood vessels, gastrointestinal tract, and kidneys. Diagnosis is based on history and physical exam findings, blood tests, autoantibodies seen in scleroderma, and imaging of heart or lungs.

What Are Common Treatments?

For Raynaud’s, blood pressure medications called calcium channel blockers or PDE-5 inhibitors like sildenafil (Viagra) and tadalafil (Cialis), can improve circulation. Antacids and proton pump inhibitors (omeprazole) can help with heartburn. There are no approved medications for skin thickening, but methotrexate (Rheumatrex) and mycophenolate mofetil (Cellcept) can be used early in the disease depending on the presence of other symptoms such as joint, muscle, or lung disease. Mycophenolate is also used to treat patients who develop scarring or inflammation of the lungs (known as interstitial lung disease). In more severe lung cases, treatment with tocilizumab, which is a biologic medication might be helpful. In patients with even more progressive disease, treatment with nintedanib (Ofev) or rituximab can be tried. Other treatments for a serious lung complication (pulmonary arterial hypertension) can relax constricted blood vessels to ease high blood pressure. In some patients with diffuse forms of scleroderma a bone marrow transplant can also be considered.

Living with Scleroderma

Patients with Raynaud’s phenomenon should keep their bodies, hands, and feet warm with layered clothing, boots and gloves in cold weather. Protect fingers and toes from activities that could injure skin. Keep skin moisturized. People with digestive problems might change their diet to prevent heartburn. Eating several smaller meals instead of three large ones, as well as not eating right before bed, can also help with heartburn. Regular exercise and physical therapy may keep joints flexible. Additionally, depression or mood problems are possible. Seek support from family or friends, patient groups, or if needed, mental health treatment from a psychologist. It is important to remember that it is possible for patients with scleroderma to lead long, fulfilling lives.

Updated February 2025 by Diana Girnita MD, PhD, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.