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Alternative Names Return to topCREST 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.