Medical Encyclopedia


Medical Encyclopedia

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Contents of this page:


Raynaud's phenomenon
Raynaud's phenomenon
CREST syndrome
CREST syndrome

Alternative Names    Return to top

CREST syndrome; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma

Definition    Return to top

Scleroderma is a widespread connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs.

Causes    Return to top

The cause of scleroderma is unknown. People with this condition have a build up of a substance called collagen in the skin and other organs. This build up leads to the symptoms associated with the disease.

The disease usually affects people 30 to 50 years old. Women get scleroderma more often than men do. Risk factors include occupational exposure to silica dust and polyvinyl chloride.

Symptoms    Return to top

Skin symptoms may include:

Bone and muscle symptoms may include:

Breathing problems may include:

Digestive tract problems may include:

Additional symptoms associated with this disease include:

Exams and Tests    Return to top

Examination of the skin may show tightness, thickening, and hardening.

Tests may include:

Treatment    Return to top

Drugs used to treat scleroderma include:

Other treatments for specific symptoms may include:

Treatment usually includes a combination of physical therapy and skin and joint protection techniques (for example, avoiding cold in the case of Raynaud's phenomenon).

Support Groups    Return to top

See: Scleroderma - resources

Outlook (Prognosis)    Return to top

In most patients, the disease slowly gets worse. People who only have skin involvement have a better outlook. Death may occur from gastrointestinal, heart, kidney, or lung involvement.

One type of scleroderma, called localized scleroderma, involves only problems of the skin of the hands and face. It gets worse very slowly and usually does not affect any other parts of the body.

Systemic scleroderma can involve many organs in the body. In some people, it will progress slowly and not involve any organs in the body. In others, organs such as the lungs, kidneys, intestines, gallbladder, and heart become involved.

For some, symptoms and problems develop quickly over the first few years, and continue to worsen. Others get worse much more slowly. Problems with the lungs are the most common cause of death in patients with scleroderma.

Possible Complications    Return to top

When to Contact a Medical Professional    Return to top

Call for an appointment with your health care provider if:

Prevention    Return to top

There is no known prevention. Minimize exposure to silica dust and polyvinyl chloride.

References    Return to top

Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007.

Harris ED Jr., Budd RC, Genovese MC, Firestein GS, Sargent JS, eds. Kelley's Textbook of Rheumatology. 7th ed. Philadelphia, Pa: Saunders Elsevier; 2005.

Clouse RE, Diamant NE. Esophageal Motor and Sensory Function and Motor Disorders of the Esophagus. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2006: chap 41.

Update Date: 2/22/2009

Updated by: Ariel D. Teitel, MD, MBA, Chief, Division of Rheumatology, St. Vincent’s Hospital, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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