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Putting the two Greek words sclero (meaning hard) and derma (meaning skin) together paints a picture of the condition known as scleroderma. In this condition, the skin becomes thicker and harder.
Scleroderma belongs to the same family of autoimmune diseases as Lupus and Rheumatoid Arthritis. It shares many features with these illnesses, including the fact that it often appears in adult women. Women are 3 to 5 times more likely to develop this condition than men are.
Another thing it has in common with these diseases is that it can cause a wide range of different symptoms and complications. For some people, it's a lifelong nuisance; for others, it's a disease that quickly worsens and may lead to death. This disease is not contagious, and it is not inherited.
About 16,000 Canadians live with scleroderma. It can affect a specific area of the body, usually the skin (called localized scleroderma). It may also affect several areas of the body, including internal organs (called systemic or generalized scleroderma).
Two forms of localized scleroderma are morphea and linear scleroderma. They usually affect only the skin, but may also affect muscles and bones.
Generalized or systemic scleroderma also has 2 forms: limited scleroderma (or CREST syndrome) and diffuse scleroderma.
Most people with scleroderma develop the condition between the ages of 30 and 50 years, although it may occur in children and seniors.
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The exact cause of scleroderma is unknown. Scleroderma is a rare autoimmune disease, a disease in which the immune system attacks its own healthy cells, causing damage.
Normally, the immune system helps the body deal with infection and injury. People with scleroderma produce too much collagen, a protein that makes up connective tissue in tendons and ligaments. Depending on the type of scleroderma a person has, the extra collagen is deposited in either the skin or in other organs in the body, or both, which causes hardening of the tissues.
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Symptoms of scleroderma include:
There are 2 types of generalized scleroderma:
The 5 main features of CREST syndrome include:
Diffuse scleroderma is the other form of systemic scleroderma. In diffuse scleroderma, people experience thickening of the skin of the arms, legs, face, chest, and stomach. Inside the body, damage to the lungs, kidneys, heart, blood vessels, bowel, esophagus, and joints may occur. If many of these organs are affected, the condition may become life-threatening. This more serious form of scleroderma is occasionally fatal, and nearly always debilitating.
The most common forms of damage from diffuse scleroderma are:
Many of the most severe complications of scleroderma are likely to occur in the first 5 years or not at all. People with severe forms of scleroderma have about an 80% to 85% chance of surviving 5 years after being diagnosed with the condition.
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There's no test that will give a guaranteed diagnosis of scleroderma while excluding other diseases. The doctor must balance the symptoms, history, and examination against the often-confusing evidence from blood tests and samples of skin to come up with an idea of what condition is present. At first, the condition may look similar to other autoimmune conditions including rheumatoid arthritis or lupus.
Once scleroderma has been diagnosed, the doctor can determine whether it is localized or generalized. The presence of certain antibodies helps to identify which type of scleroderma is present.
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There is no cure for scleroderma. However, there are medications that can reduce symptoms and organ damage.
Limited scleroderma is usually treated with topical (applied to the skin) treatments such as moisturizers or topical corticosteroid creams. Generalized scleroderma (involving the internal organs) may be treated with medications that improve blood flow, promote esophagus and bowel function, keep the kidneys functioning, and control high blood pressure.
Here are some recommendations to reduce the risk of problems caused by Raynaud's phenomenon:
Medication treatment with calcium channel blockers may be prescribed to treat Raynaud's phenomenon. They work by causing blood vessels to expand, and this improves blood flow in the skin.
Symptoms such as joint stiffness and pain can be relieved with anti-inflammatory medications such as acetylsalicylic acid* (Asa), NSAIDs (non-steroidal anti-inflammatory drugs), or corticosteroids. Certain medications called DMARDs (disease modifying antirheumatic drugs) are often prescribed along with NSAIDs. Both help reduce joint inflammation. Another class of medications called immunosuppressants may be used to suppress the immune system and prevent inflammation.
For digestive problems associated with scleroderma, antacids and antibiotics (as prescribed by a doctor) may be helpful. The classes of medications known as H2 blockers and proton pump inhibitors can help reduce damage due to gastroesophageal reflux. The esophagus can begin to narrow, and may have to be physically stretched from time to time with an inflatable tube device called a balloon catheter.
Antibiotics can prevent bacterial infection of damaged parts of the intestines.
Kidney problems used to be the major cause of death in scleroderma, but medications called ACE inhibitors can help prevent life-threatening high blood pressure caused by kidney damage related to scleroderma. In the case of complete renal (kidney) failure, a dialysis machine may be needed.
Interstitial lung disease (scarring of lung tissue) can be treated with immunosuppressant drugs. Pulmonary hypertension (high blood pressure in the lungs) can be controlled with drugs that reduce blood pressure in the lungs such as Epoprostenol, Bosentan, Riociguat, and Sildenafil.
Although there is no cure for scleroderma, there is ongoing research focusing on medications that would slow the progression of the disease and ease symptoms.
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