Close

Scleroderma

Alternate Names

  • progressive systemic sclerosis

Definition

Scleroderma is a widespread autoimmune disorder. It causes the skin and other body parts to slowly degenerate, thicken, and stiffen. An autoimmune disorder is one in which the body produces antibodies against its own tissues for no known reason.

What is going on in the body?

Scleroderma results from an overproduction of collagen, which is the main supportive protein in the body. The excess collagen affects tiny blood vessels and almost every organ.
A person may develop either a localized or a systemic form of scleroderma. The localized form is most common in children. It usually involves a few places on the skin, soft tissues, or muscles. The localized form rarely, if ever, progresses to the systemic form of scleroderma.
The systemic form affects skin, muscles, and soft tissues. It also affects other body organs and structures, such as the following:
  • blood vessels
  • gastrointestinal (GI) tract
  • heart
  • kidneys
  • lungs

Risks

What are the causes and risks of the condition?

The exact cause of scleroderma is unknown. People heavily exposed to certain chemicals or substances may be at higher than normal risk for scleroderma. These substances include the following:
  • detergent
  • herbicides
  • plastics
  • silica
  • silicone prostheses
  • various drugs, such as cocaine and amphetamines
Mild cases may develop from occupational repetitive stresses on the hand, such as from working with a jackhammer. This condition usually occurs between the ages of 30 and 50, but it may also occur in children. It is four times more common in women than men but can affect either sex.

Prevention

What can be done to prevent the condition?

There is no known way to prevent scleroderma.

Diagnosed

How is the condition diagnosed?

The diagnosis of scleroderma begins with a medical history and physical exam. Raynaud's phenomenon may be an early sign of scleroderma. A skin biopsy may help diagnose scleroderma. A tissue sample of the affected skin is removed for laboratory study. The healthcare professional may also order tests of heart, lung, and GI tract function.

Long Term Effects

What are the long-term effects of the condition?

There is no cure for scleroderma at this time. In a minority of people with the condition, problems involving the organs can cause:
  • high blood pressure
  • kidney failure
  • lung problems
  • malnutrition
Death may result from gastrointestinal, heart, kidney, or lung involvement.

Other Risks

What are the risks to others?

Scleroderma is not contagious and poses no risk to others.

Treatments

What are the treatments for the condition?

Treatment depends on the symptoms and severity of the scleroderma. Following is a list of some of the medications used to treat this disorder:
  • angiotensin-converting enzyme (ACE) inhibitors to relax and dilate blood vessels
  • antacids and other medications to reduce heartburn
  • antibiotics to fight infection
  • anti-rheumatic medications such as hydroxychloroquine (i.e., Plaquenil) or d-penicillamine. These reduce skin thickening and lung problems.
  • calcium channel blockers to treat Raynaud's phenomenon
  • corticosteroids, such as prednisone (i.e., Sterapred), to reduce inflammation
  • immunosuppressive medications, such as methotrexate (i.e., Rheumatrex, Trexall), azathioprine (i.e., Imuran, Azasan), cyclophosphamide (i.e., Cytoxan), and cyclosporine (i.e., Sandimmune, Neoral, Gengraf). These suppress the immune response to reduce inflammation.
  • nitroglycerin ointment (i.e., Nitro-Bid) to treat hardened skin
  • NSAIDs, such as ibuprofen (i.e., Advil, Motrin) and aspirin, to reduce inflammation
Psychotherapy or counseling may help the person adjust to living with an incurable disease. There are support groups for this disorder. Joining one may help the person to deal with the illness.

Side Effects

What are the side effects of the treatments?

Medications used to treat scleroderma have many side effects. The side effects include the following:
  • Antibiotics may cause stomach upset, diarrhea, and allergic reactions.
  • Corticosteroids may cause weight gain, high blood pressure, bone loss or osteoporosis, an increased risk of infection, and muscle weakness.
  • Antibiotics may cause stomach upset, diarrhea, and allergic reactions.
  • Medications that suppress the immune system may cause an increased risk of infection, stomach upset, and liver or kidney damage.
  • NSAIDs can cause stomach upset and a decrease in kidney function.
Calcium channel blockers have long been used to treat scleroderma as well as high blood pressure. However, the findings of two recent studies show that people who take a calcium channel blocker have a much higher incidence of complications than people taking other medications for high blood pressure. The findings of one study, for example, showed that the risk of heart attack was 27% greater. The risk of congestive heart failure was 26% higher in people taking calcium channel blockers. The American Heart Association recommends discussing the risks and benefits of the medication with a healthcare professional.

After Treatment

What happens after treatment for the condition?

The course of scleroderma varies unpredictably. A person who only has the local form of scleroderma has a better prognosis. An individual with severe systemic scleroderma may have chronic lung, kidney, gastrointestinal, and cardiovascular problems.

Monitor

How is the condition monitored?

A person with scleroderma should have frequent physical exams by his or her healthcare professional. This helps him or her monitor the activity of the disorder and determine possible complications. Frequent blood tests may help monitor the disorder as well. Any new or worsening symptoms should be reported to the healthcare professional.

Sources

The Merck Manual of Medical Information, Home edition, 1997

Professional Guide to Diseases, Sixth Edition. Springhouse: Springhouse Corporation, 1998.

Tierney, Lawrence, editor, "Current Medical Diagnosis and Treatment, 39th edition", 2000.

Griffith, H. Winter. Instructions for Patients. Philadelphia:W.B. Saunders company,1994

Understanding Autoimmune Disorders, [hyperLink url="http://www.niaid.nih.gov/publications/autoimmune.htm" linkTitle="www.niaid.nih.gov/publications/autoimmune.htm"]www.niaid.nih.gov/publications/autoimmune.htm[/hyperLink]

Leverage our buying power. We serve GOV/EDU/MIL, employers, facilities & inventors.

  Account set up & astute solutions guidance.
  Free edu catalogs for your staff & community.

Engage in our innovative medical & health care, sales, education & marketing programs.

  Publishing, marketing & lead gen programs.
  Vendors/Investors: full-service solutions.

Get Discovery Digest e-news updates, discounts, and exclusive offers.

  
  EZ Publish our Discovery Digest free.