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Scleroderma

Sclerodactyly
Telangiectasia

Definition

Scleroderma is a disease that involves the buildup of scar-like tissue in the skin and elsewhere in the body. It also damages the cells that line the walls of the small arteries.

Alternative Names

Progressive systemic sclerosis; Systemic sclerosis; Limited scleroderma; CREST syndrome; Localized scleroderma; Morphea - linear; Raynaud's phenomenon - scleroderma

Causes

Scleroderma is a type of autoimmune disorder. In this condition, the immune system mistakenly attacks and damages 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. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.

Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis. These cases are referred to as undifferentiated connective tissue disease or overlap syndrome.

Symptoms

Some types of scleroderma affect only the skin, while others affect the whole body.

Skin signs of scleroderma may include:

Bone and muscle symptoms may include:

Breathing problems may result from scarring in the lungs and can include:

Digestive tract problems may include:

Heart problems may include:

Kidney and genitourinary problems may include:

Exams and Tests

The health care provider will do a complete physical exam. The exam may show:

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.

Blood and urine tests may include:

Other tests may include:

Treatment

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:

Other treatments for specific symptoms may include:

Treatment often involves physical therapy as well.

Support Groups

Some people can benefit from attending a support group for people with scleroderma.

Outlook (Prognosis)

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.

When to Contact a Medical Professional

Call your provider if you develop Raynaud phenomenon, progressive thickening of the skin, or trouble swallowing.

References

Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327-1339. PMID: 27941129 www.ncbi.nlm.nih.gov/pubmed/27941129.

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(6):1241-1247. PMID: 22467932 www.ncbi.nlm.nih.gov/pubmed/22467932.

Varga J. Etiology and pathogenesis of scleroderma. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley and Firestein's Textbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017:chap 83.

Varga J. Systemic sclerosis (scleroderma). In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 267.


Review Date: 10/1/2017
Reviewed By: 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, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.