• Scleroderma

    (Systemic Sclerosis)

    Definition

    Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are three major forms of the disease:
    • Localized scleroderma (also known as morphea)—usually affects only the skin in isolated parts of the body. This form is less serious.
    • Systemic scleroderma—affects widespread areas of skin and/or internal organs, most often the lungs (Certain categories of this form of scleroderma are more serious and can be fatal.)
    • Overlap syndrome—may involve features of scleroderma and features of other autoimmune syndromes

    Causes

    Overproduction of collagen and other connective tissue proteins is the main feature of scleroderma. It is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production. There is also evidence that scleroderma may result from vascular abnormalities.

    Risk Factors

    These factors increase your chance of developing scleroderma. Tell your doctor if you have any of these risk factors:
    • Sex: Female
    • Age: 30-50 years
    • Occupational exposures (such as, polyvinyl chloride or silica dust)
    • Genetic predisposition

    Symptoms

    Localized Scleroderma

    Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:
    • Hard patches on the skin, most often on the face or trunk (morphea)
    • Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)

    Systemic Scleroderma

    This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with Raynaud's phenomenon. This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.
    Raynaud's Phenomenon Symptom
    Low blood flwo to fingers, vasoconstriction
    Copyright © Nucleus Medical Media, Inc.
    Diffuse scleroderma comes on more suddenly and can progress to involve not only the skin but the internal organs. Other symptoms of diffuse scleroderma may include:
    • Diffuse thickening and hardening of the skin
    • Joint and muscular pain, stiffness, and swelling
    • Problems with breathing, swallowing, and digesting food due to thickening and hardening of lung, esophagus, bowel tissues
    • Inflammation and thickening of large and small blood vessels
    Complications of diffuse scleroderma can affect virtually every system of the body. Prominent complications include:

    Diagnosis

    The doctor will ask about your symptoms and medical history, and perform a physical exam. Diagnosis is based on changes in the skin. Other tests may include:
    • Blood tests
    • Esophagus motility study
    • X-ray—a test that uses radiation to take a picture of structures inside the body, especially bones
    • MRI scan—a test that uses magnetic waves to make pictures of structures inside the body
    • CT scan—a type of x-ray that uses a computer to make pictures of structures inside the body
    • Biopsies of skin and other tissues—removal of a sample of tissue to test

    Treatment

    There is no treatment to cure scleroderma. Treatments are aimed at relieving symptoms.

    Joints and Muscles

    • Aspirin
    • Nonsteroidal anti-inflammatory drugs
    • Corticosteroids, or prednisone (Deltasone, Orasone, Prednicen-M)

    Esophagus and Digestive Tract

    • Medicines to limit acid production in the stomach
    • Small, frequent meals
    • Sleeping with your head elevated to avoid acid reflux

    Skin and Internal Organs

    Kidney Problems and/or High Blood Pressure

    Raynaud's Phenomenon

    • Calcium channel blockers, like nifedipine (Adalat, Procardia, Afeditab, Nifediac)—to dilate blood vessels in the extremities
    • Proper shelter and clothing to avoid cold
    • Not smoking

    Restricted Mobility

    Prevention

    There are no guidelines for preventing scleroderma because the cause is unknown.

    RESOURCES

    Scleroderma Foundation http://www.scleroderma.org

    Scleroderma Research Foundation http://www.srfcure.org

    CANADIAN RESOURCES

    Arthritis Society http://www.arthritis.ca

    Scleroderma Society of Canada http://www.scleroderma.ca

    References

    Arthritis Foundation website. Available at: http://www.arthritis.org. Accessed July 9, 2009.

    Firestein ED et al. Kelley’s Textbook of Rheumatology. 8th ed. Philadelphia: Saunders, 2008.

    Habif TP. Clinical Dermatology. 4th ed. St. Louis: Mosby, 2004.

    Kreuter A, Hyun J, et al. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2006;54:440-447.

    Mathai SC, Girgis RE, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007;29:469-475.

    Mayo Foundation for Medical Education and Research website. Available at: http://www.mayo.edu. Accessed July 9, 2009.

    The Merck Manual of Medical Information. 17th ed. Simon and Schuster, Inc; 2000.

    Rodriguez-Reyna TS, Alarcon-Segovia D. Overlap syndromes in the context of shared autoimmunity. Autoimmunity. 2005;38:219-223.

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