Scleroderma is a type of autoimmune disorder. In this condition, the immune system mistakenly attacks and destroys healthy body tissue.
The cause of scleroderma is unknown. A buildup of a substance called collagen in the skin and other organs leads to the symptoms of the disease.
The disease most often affects people 30 to 50 years old. Women get scleroderma more often than men do. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.
The health care provider will do a physical exam. The exam may show tight, thick skin.
Your blood pressure will be checked. Scleroderma can cause small blood vessels in the kidneys to become narrowed. Problems with your kidneys can lead to high blood pressure and decrease function of the kidney.
There is no specific treatment for scleroderma. Your provider will assess the extent of disease in the lungs, kidneys, heart, and gastrointestinal tract.
You will be prescribed medicines and other treatments to control your symptoms and prevent complications.
Medicines used to treat scleroderma include:
Corticosteroids such as prednisone. Doses above 10 mg per day are not recommended.
Drugs that suppress the immune system such as methotrexate
Nonsteroidal anti-inflammatory drugs (NSAIDs)
Other treatments for specific symptoms may include:
Medicines for heartburn or swallowing problems
Blood pressure medicines (such as ACE inhibitors) for high blood pressure or kidney problems
Light therapy to relieve skin thickening
Medicines to improve breathing (bosentan)
Treatments to improve Raynaud phenomenon, including medicines, gloves to keep the hands warm, and avoiding smoking
Treatment often involves physical therapy as well.
Some people can benefit from attending a support group for people with scleroderma.
In some people, symptoms develop quickly for the first few years and continue to get worse. However, in most people, the disease gets worse slowly.
People who have only skin symptoms have a better outlook. Widespread (systemic) scleroderma can lead to.
Scarring of the lungs, called pulmonary fibrosis
High blood pressure in the lungs (pulmonary hypertension)
Problems absorbing nutrients from food
When to Contact a Medical Professional
Call your health care provider if your symptoms become worse or new symptoms develop.
Mendoza FA, Nagle SJ, Lee JB, Jimenez SA. A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. J Rheumatol. 2012; 39:1241. PMID: 22467932 www.ncbi.nlm.nih.gov/pubmed/22467932.
Varga J. Etiology and Pathogenesis of Scleroderma. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 83.
Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.