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Scleroderma
Definition
Scleroderma is a widespread connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs.
Alternative Names
CREST syndrome; Progressive systemic sclerosis; Systemic sclerosisCauses
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
- Blanching, blueness, or redness of fingers and toes in response to heat and cold (Raynaud's phenomenon)
- Bloating after meals
- Constipation
- Diarrhea
- Difficulty swallowing
- Esophageal reflux or heartburn
- Pain, stiffness, and swelling of fingers and joints
- Shortness of breath
- Skin thickening and shiny hands and forearm
- Skin is hard
- Tight and mask-like facial skin
- Ulcerations on fingertips or toes
- Weight loss
- Eye burning, itching, and discharge
- Hair loss
- Joint pain
- Skin, abnormally dark or light
- Wheezing
- Wrist pain
Exams and Tests
Examination of the skin may show tightness, thickening, and hardening.
Tests may include:
- Antinuclear antibody
- Chest x-ray
- CT scan of the lungs
- Echocardiogram
- ESR
- Rheumatoid factor
- Urinalysis
- Pulmonary function studies
- Skin biopsy
Treatment
Drugs used to treat scleroderma include:
- Corticosteroids
- Nonsteroidal anti-inflammatory drugs (NSAIDs)
- Immunosuppressants (Methotrexate, Cytoxan)
Other treatments for specific symptoms may include:
- Antacids for heartburn
- Blood pressure medications (particularly ACE inhibitors) for high blood pressure or kidney problems
- Medicines to improve breathing
- Medications to treat Raynaud's phenomenon
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
See: Scleroderma - support group
Outlook (Prognosis)
In most patients, the disease slowly gets worse. People who only have skin involvement have a better outlook. Death may occur from gastrointestinal, cardiac, kidney, or pulmonary (lung) involvement.
Possible Complications
- Heart failure
- Kidney failure
- Malabsorption
- Pulmonary fibrosis
- Pulmonary hypertension
When to Contact a Medical Professional
Call for an appointment with your health care provider if:
- You have symptoms of scleroderma
- You have scleroderma and symptoms become worse or new symptoms develop
Prevention
There is no known prevention. Minimize exposure to silica dust and polyvinyl chloride.
References
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.
Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, New York, NY, and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by Verimed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
