The exact cause of scleroderma is not known, but there are multiples theories. Changes in the body's immune system may cause the immune cells to react to the body. This autoimmune reaction causes the stimulation of fibroblast cells (cells that make up collagen and other connective tissues). The result is the excessive collection of collagen and other connective tissue components in various parts of the body such as the skin, lungs and walls of the arteries. This overproduction of collagen causes the thickening and hardening of the skin and other organs.
Another theory implicates the vascular system. It is suspected that an unknown triggercauses an injury to the cells that line the small arteries. Such injury to the blood vessels may cause them to constrict and stiffen, resulting in overreaction to cold and/or stress. As a consequence there may be damage to the vessels themselves and possibly to organs.
Another study into the causes of scleroderma is investigating a virus-like trigger. Similarly other studies are looking at possible environmental impacts, suggesting that there may be a genetic predisposition to the disease with an environmental trigger.
While the cause of scleroderma is not known it is believed that scleroderma is neither contagious, nor inherited.
Yes. The Ontario's Workplace Safety and Insurance Board recognizes scleroderma resulting from occupational exposure to silica dust as an occupational disease pursuant to subsection 2(1) of the Workplace Safety and Insurance Act.
Currently, scleroderma is classified as a chronic, progressive disease with no known cure. As such, it is important to receive a proper diagnosis, be monitored and treated by the appropriate health professional in order to minimize symptoms and lessen the chance of irreversible damage.
Scleroderma is not considered a hereditary disease. However, it is believed that families with a history of rheumatic diseases may be predisposed.
Regular self-monitoring of blood pressure is critical in newly-diagnosed people. Increases in blood pressure signify the onset of hypertension. Hypertension requires rapid action by medical professionals as it may indicate renal crisis. Renal crisis is a medical emergency and considered one of the most life- threatening complications of scleroderma. People recently diagnosed with diffuse scleroderma are at a much higher risk of developing renal crisis compared to those with limited scleroderma.
Although there is a single disease called scleroderma, one person’s symptoms and course may look very different from another’s. Similarly, people do many different things to cope and live as well as possible with scleroderma, and some strategies may work well for one person and not for another. Generally, successful coping strategies fall into three areas:
(1) Obtain accurate information on scleroderma and develop relationships with knowledgeable and caring healthcare professionals in order to get the best possible care.
There are several publications that people with scleroderma and people close to them have found to be helpful in understanding and coping with the disease.
(2) Developing and maintain a good support network.
Beyond family members and friends, consider support from other people living with scleroderma; professional supports, such as psychologists or therapists, and/or spiritual support.
(3) Staying as active and involved as possible.
Although staying active and involved may require making adaptations to usual activities or seeking alternative activities, research supports the physical and emotional benefits.
Compared to approximately 5% of people in the general population who have depression at any given time, the rate may be as high as 15% among people with scleroderma. Although most people with scleroderma do not have depression, many experience other forms of emotional distress, such as bouts of sadness, frustration, and anxiety or worry related to living with the condition.
Absolutely. There have been huge advances in the treatment of symptoms of scleroderma in the last decade. Survival rates amongst those diagnosed are much improved as well as patient’s quality of life. Fortunately, Scleroderma research has become a very popular field in the last decade and there are many promising studies being conducted at this time.
Yes. Smoking is not a healthy choice for anyone, but for those suffering from scleroderma, smoking can exacerbate the respiratory, vascular and gastrointestinal systems. Stopping smoking is the number one thing you can do to lessen your symptoms and improve your longevity. Smoking constricts blood vessels and can impact Raynauds symptoms. Smoking also makes your mouth dry which can worsen the symptoms of Sjorgens Syndrome.
Digital ulcers are skin sores that most often occur on your fingers, but are also reported on toes and joints. These ulcers are extremely painful and are difficult to heal. They occur because there is limited blood circulation. Ulcers often start as a cut that doesn’t heal properly. Preventing and healing ulcers can be difficult.
Raynaud's Phenomenon occurs when the blood vessels in the fingers and toes contract excessively in response to cold and stress. The resulting reduction in blood flow causes fingers (and toes) to become blue or white in colour and may be accompanied by pain and numbness. Wear multiple thin layers of clothing. Ensure that layers are not too tight so they do not restrict circulation. Stress management should be another goal.
A recent survey of more than 400 Canadians with scleroderma found that 76% surveyed reported difficulty sleeping at least some of the time and 59% said that poor sleep affected their ability to function at least moderately. Difficulty with sleep may be affected by cigarette smoking, alcohol and coffee consumption, and many prescription medications. Depression, anxiety, and worry have also been linked to poor sleep. For many people living with scleroderma, pain can play a major role in sleep problems. Gastrointestinal problems, including heartburn, can also be uncomfortable and painful, making sleep difficult.
Fatigue from scleroderma is different from normal tiredness and may become debilitating. Of the more than 400 Canadians with scleroderma surveyed by the Scleroderma Society of Canada/Canadian Scleroderma Research Group, almost 90% said they experienced fatigue at least some of the time, and more than 70% said that it had a moderate to severe impact on their ability to carry out normal daily activities. Light, consistent exercise may help keep the body strong and boost energy. Similarly, eating a nutritional diet and maintaining a healthy weight can help combat fatigue as will getting enough rest, pacing oneself through the course of the day to avoid overexertion, and managing stress.
Heartburn in scleroderma patients is caused by the dysfunction of the valve that separates the stomach and the esophagus. As a result stomach acid flows backward up the esophagus causing heartburn. Abnormal contractions of the esophagus also occur so acid is not cleared properly and may result in difficulty swallowing. Strategies for coping with heartburn include using over-the-counter medications that neutralize or reduce stomach acids; elevating the head of your bed; eating smaller meals, and avoiding foods that cause heartburn. Chewing food well, eating slowly, as well as drinking fluids with meals may also reduce heartburn.
People with scleroderma have thickened and tight skin which can lead to stiffness and reduced motion of the joints in affected areas. Some studies have shown that those with scleroderma may benefit by range of motion exercises, where the fingers, hands and wrists are stretched daily to keep them moving and mobile. Other studies have found connective tissue massage with joint manipulation may also be effective in combating stiffness.
Sexual dysfunction can be an issue. Men with scleroderma suffer more frequently from erectile dysfunction compared to the general population. Treatment may be a challenge as there is limited research into effective treatment.
Dry mouth, also called Sjogren’s Syndrome, is a less than desirable symptom of scleroderma. Stay hydrated by constantly sipping beverages, sucking on hard candy or chewing gum. Avoid foods that dehydrate the mouth and body such as alcohol, caffeine, or salty foods. When eating, it may be difficult to swallow, in which case drinking liquids may help.
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