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 3 major forms of the disease:
  • Localized scleroderma (can be morphea or linea)—Usually affects only the skin in isolated parts of the body. This form is less serious.
  • Systemic sclerosis—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 are the main features of scleroderma. It is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production.

Risk Factors

Scleroderma is more common in women. The morphea type of scleroderma usually affects people 20-40 years old. Linear scleroderma often occurs in children. Systemic scleroderma is more likely to occur in people aged 30-50 years old.Other factors that may increase your risk of developing scleroderma include:
  • Family members with autoimmune disorders
  • Occupational chemical exposure, such as silica, ketones, or welding fumes

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 plaques or thickened skin.
  • Thickening can extend to underlying muscles and bones (linear scleroderma or linear morphea)
  • Muscle and joint aches, and tiredness may occur with 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. The fingers turn red after resolution of the symptoms. 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
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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. Other common complications include:

Diagnosis

You will be asked about your symptoms and medical history. A physical exam will be done. Diagnosis is based on changes in the skin.Tests may include:
  • Blood tests
  • Biopsies of skin and other tissues
Imaging tests take pictures of internal body structures. These may include:In addition, an esophagus motility study may be done

Treatment

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

Joints and Muscles

  • Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen
  • Corticosteroids

Esophagus and Digestive Tract

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

Skin and Internal Organs

  • Corticosteroids
  • Immunosuppressants
  • Prostanoids—drugs that control the immune system
  • Phototherapy

Kidney Problems and/or High Blood Pressure

  • ACE inhibitors, or other antihypertensive drugs
  • Antineoplastic agents
  • Vasodilators

Raynaud's Phenomenon

  • Calcium channel blockers
  • Proper shelter and clothing to avoid cold
  • Prostanoids—drugs that control the immune system
  • If you smoke, talk to your doctor about ways to quit.

Restricted Mobility

Physical therapy and exercise are important to maintain circulation, joint flexibility, and muscle strength .

Prevention

There are no current guidelines to prevent scleroderma.

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

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.

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

Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases website. Available at: http://www.niams.nih.gov/Health%5FInfo/Scleroderma/default.asp. Accessed January 15, 2015.

Systemic sclerosis. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated September 8, 2014. Accessed January 15, 2015.

What is scleroderma? Scleroderma Foundation website. Available at: http://www.scleroderma.org/site/PageNavigator/patients%5Fwhatis.html. Accessed January 15, 2015.

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