|
 |
|
|
|
|
|
scleroderma Alternative 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 localised or a systemic form of scleroderma. The localised form is most common in children. It usually involves a few places on the skin, soft tissues, or muscles. The localised 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, or GI, tract
- heart
- kidneys
- lungs
What are the signs and symptoms of the condition?
Scleroderma can progress slowly, over many years. It often starts with the appearance of Raynaud's phenomenon. This condition causes abnormal sensitivity to cold in the fingers and toes. Swelling might be noted in the hands.
Sweat secretion is decreased over involved areas of skin. The skin becomes increasingly hard and dry. Extremities stiffen, and mobility is decreased. The mouth can become rigid. The face may look masklike and immobile. Body image can be negatively affected. Changes not visible inside the body are important and threatening.
Following are some additional signs and symptoms of scleroderma: - calcium deposits in the skin and muscles
- digestive problems, such as trouble swallowing, poor food absorption, bloating after eating, weight loss, and heartburn
- dilated blood vessels, especially on the hands, face, and chest
- headaches
- high blood pressure
- joint pain or stiffness
- malaise, or a vague feeling of illness
- puffy hands and feet, particularly in the morning
- shortness of breath
- Sjogren syndrome, an autoimmune disorder that causes dry eyes and mouth
- slow hardening and tightening of the skin, especially on the arms, face, and hands
- sores or ulcers, especially on the fingers and toes, due to poor circulation
- swelling in the legs and ankles
- weakness
What are the causes and risks of the condition?
The exact cause of scleroderma is unknown. It is generally thought to be an autoimmune disorder.
New research findings suggest that autoimmune disorders may be triggered by a transfer of cells between the foetus and the mother during pregnancy. Women with scleroderma have more foetal cells in their blood decades after a pregnancy than women who don't have scleroderma. While further research is needed to substantiate these findings, the study does offer an explanation for the much higher incidence of autoimmune disorders in women than in men.
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.
What can be done to prevent the condition?
There is no known way to prevent scleroderma.
How is the condition diagnosed?
The diagnosis of scleroderma begins with a medical history and physical examination. 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 doctor may also order tests of heart, lung, and GI tract function.
What are the long-term effects of the condition?
There is no cure for scleroderma at this time. In a minority of people, problems involving the organs can cause: Death may result from gastrointestinal, heart, kidney, or lung involvement.
What are the risks to others?
Scleroderma is not contagious and poses no risk to others.
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: - ACE inhibitors to relax and dilate blood vessels
- antacids and other medications to reduce heartburn
- antibiotics to fight infection
- antirheumatic medications such as hydroxychloroquine or d-penicillamine. These reduce skin thickening and lung problems.
- calcium channel blockers to treat Raynaud's phenomenon
- corticosteroids, such as prednisone, to reduce inflammation
- immunosuppressive medications, such as methotrexate, azathioprine, cyclophosphamide, and cyclosporine. These suppress the immune response to reduce inflammation.
- nitroglycerin ointment to treat hardened skin
- NSAIDs, such as ibuprofen and aspirin, to reduce inflammation
Psychotherapy or counselling 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.
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, diarrhoea, 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, diarrhoea, 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.
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.
How is the condition monitored?
A person with scleroderma should have frequent physical examinations by his or her doctor. This helps the doctor 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 doctor.
Author: Gail Hendrickson, RN, BS
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request |
|
|
| |
|
|
|
|
|
|
|
|
