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What is Scleroderma?

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Scleroderma (also known as systemic sclerosis) is a disease affecting the skin and other organs of the body. Scleroderma is one of the autoimmune rheumatic diseases, meaning that the body’s immune system is acting abnormally. The main finding in scleroderma is thickening and tightening of the skin and inflammation and scarring of many body parts leading to problems in the lungs, kidneys, heart, intestinal system and other areas. There is still no cure for scleroderma but effective treatments for some forms of the disease are available.

Scleroderma – The Facts

  • Scleroderma is relatively rare. Only 75,000 to 100,000 people in the US have it. More than 75 percent of people with scleroderma are women.
  • The condition affects adults and children, but it is most common in women aged 30 to 50.
  • There are several types of scleroderma and related diseases and the names can be confusing. The two main types are localized (which affects the skin on the face, hands and feet) and systemic (which can also affect blood vessels and major internal organs).
  • Although the underlying cause is unknown, promising research is shedding light on the relationship between the immune system and scleroderma.

Scleroderma causes the skin to become thick and hard; and also results in a buildup of scar tissue; and damage to internal organs such as the heart and blood vessels, lungs, stomach and kidneys. The effects of scleroderma vary widely and range from minor to life-threatening, depending on how widespread the disease is and which parts of the body are affected.

The two main types of scleroderma are:

  • Localized scleroderma, which usually affects only the skin, although it can spread to the muscles, joints and bones. It does not affect other organs. Symptoms include discolored patches on the skin (a condition called morphea); or streaks or bands of thick, hard skin on the arms and legs (called linear scleroderma). When linear scleroderma occurs on the face and forehead, it is called en coup de sabre.
  • Systemic scleroderma, which is the most serious form of the disease, affects the skin, muscles, joints, blood vessels, lungs, kidneys, heart and other organs.

Treating Scleroderma

So far, there is no drug that has been clearly proven to stop, or reverse, the key symptom of skin thickening and hardening in Scleroderma. Medications that have proven helpful in treating other autoimmune diseases, such as rheumatoid arthritis and lupus, usually don’t work for people with scleroderma. Doctors aim to curb individual symptoms and prevent further complications with a combination of drugs and self-care. For example:

Raynaud’s phenomenon can be treated with drugs such as calcium channel blockers or drugs called PDE-5 inhibors – sildenafil (Viagra®), tadalafil (Cialis®) – which open up narrowed blood vessels and improve circulation. To prevent further damage, it’s important to keep the whole body warm, especially fingers and toes. It’s also important to protect fingertips and other skin areas from injury, which can happen even during normal daily activities.

Heartburn (acid reflux) can be treated with antacid drugs, especially proton-pump inhibitors (omeprazole and others). These medications ease gastro-esophageal reflux disease (known as GERD).

Scleroderma kidney disease can be treated with blood pressure medications called “angiotensin converting enzyme inhibitors” (ACE inhibitors). These can often effectively control kidney damage if started early and use of these drugs has been a major advance for treating scleroderma.

Muscle pain and weakness can be treated with anti-inflammatory drugs such as glucocorticoids (prednisone), intravenous immunoglobin (IVIg), and/or immunosuppressive medications. Physical therapy may be useful to maintain joint and skin flexibility.

Lung damage. There are two types of lung disease that patients with scleroderma may develop. The first type is called interstitial lung disease (scarring). There is evidence that cyclophosphamide is somewhat effective in treating the interstitial lung disease in scleroderma. Clinical trials are underway assessing the effectiveness of several other drugs for this problem.

The second type of lung disease seen in scleroderma is pulmonary arterial hypertension (high blood pressure in the arteries in the lungs). In the last 10 years, a number of drugs have become available to treat this condition, including prostacyclin-like drugs (epoprostenol, treprostinol, iloprost), the endothelin receptor antagonists (bosentan, ambrisentan), and PDE-5 inhibitors (sildenafil, vardenafil, tadalafil).

Much research is ongoing into new treatments for scleroderma. Patients and their families should know that experts remain optimistic and take comfort in the fact that work towards a cure will continue.

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This web site is run by an Arthritis Specialist based in Dubai, United Arab Emirates. On this site you will find news about the latest in arthritis, information about research results in the field, tips and information and diet and exercise, and much more.



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