Q: What causes Scleroderma?
A: The cause of Scleroderma is currently unknown. Scientific evidence shows that scleroderma is not contagious, not infectious and not cancerous, while studies of twins also suggest that scleroderma is not inherited. It can affect men and women of all races and ethnic groups at any stage of life, from infancy to late adulthood. Recent research indicates there may be some external factors that can trigger scleroderma in people who are predisposed to the disease. There is also some research suggesting viral and bacterial infections may start the development of the disease.
Some environmental factors have now been identified as potential triggers for the development of a scleroderma-like condition. Some workers in the plastics industry using polyvinyl chlorides (PVC) have developed a scleroderma-like condition. Exposure to PVC may be occupational, or through a variety of ordinary items like car interiors, water pipes, plastic food wrappers, and carpets. Everyday exposure levels may be enough to induce ‘multiple chemical sensitivity syndrome’ in some people. Other substances such as silica, and epoxy resins can also cause a condition which has the features of scleroderma. There is a good deal of evidence that silicone implants or occupational exposure to silica can cause the disease, and patients with autoimmune disorders such as scleroderma sometimes dramatically improve when their silicone implants are removed. Solvents such as those found in typewriter correction fluid, paint removers, and other products have also been linked to scleroderma. Contaminated rapeseed oil, sold as olive oil in Spain in the early 1980's resulted in many people dying of a condition called ‘toxic oil syndrome’. Some of the survivors of this condition went on to develop a scleroderma type illness.
Scleroderma caused by environmental factors appears to respond better to treatment than scleroderma from an unknown cause. Doctors have traditionally believed that the disease occurs spontaneously and cannot be cured, but a growing number of experts suspect an autoimmune response to environmental toxins. Although some environmental triggers have been identified, it is important to recognize that for most people with scleroderma, the cause is unknown, and environmental factors may not be involved at all.*
Q: Is Scleroderma inherited?
A: Scleroderma is not considered a hereditary disease, however, some families are more affected by autoimmune disorders than others. Although scleroderma is not directly inherited, some scientists feel there is a slight predisposition to it in families with a history of rheumatic diseases. When examining the role inheritance may play in acquiring scleroderma, three main factors come into play: family history, genetics and ethnicity. A family history of the disease is the strongest risk factor for scleroderma, but even among family members, the risk is very low at less than 1%. Genetic factors appear to play a role in triggering the disease, but most cases are unlikely to be inherited. Preliminary research suggests that patients with certain gene variations may be more susceptible to scleroderma than those who do not carry these variations. Finally, limited data suggests that ethnicity may also play a role. African Americans and Native Americans generally have more severe scleroderma than Caucasians. These findings suggest a hereditary or genetic component to scleroderma and other autoimmune disorders. Studies suggest that the risk is highest for Choctaw Native Americans, followed by African-Americans, Hispanics, Caucasians and finally Japanese Americans.*
Q: How serious is Scleroderma?
A: Currently, Scleroderma is classified as a chronic disease with no known cure. The diagnosis of any chronic disease is considered serious. Scleroderma has a wide range of symptoms which are specific to each patient, making it difficult to generalize how serious this disease is. The severity will depend a great deal on which parts of the body are affected and to what extent. Proper diagnosis, monitoring and treatment by physicians can minimize symptoms of scleroderma and lessen the chance of irreversible damage.
Q: Is there any hope for a cure for Scleroderma?
A: Yes, there is a great deal of hope for improved outcomes in people diagnosed with scleroderma. The past decade has seen huge advances symptom treatment and management. Survival rates amongst those diagnosed are much improved as is the quality of life. Fortunately, scleroderma research has become a very popular field and there are many promising studies being conducted at this time.
Q: How do I cope with Scleroderma?
A: Since everyone living with scleroderma has a unique experience with a varying degree of severity and spread of symptoms, each person’s prognosis and coping mechanisms are different. People take many different actions to cope with scleroderma, and some strategies may work well for one person yet not as well for another. There are however, some guiding principles that have proven immensely effective in helping people that are living with the disease:
Knowledge: knowledge is power. Obtaining abundant and accurate information on scleroderma is critical. Read everything available, beginning with the information on this website and partner organizations.
Medical care: developing relationships with knowledgeable and caring healthcare professionals ensures receiving the best possible care within the medical system
Support networks: developing and maintaining strong support networks to assist in providing practical and emotional support. Support can come from many sources including family, friends, support networks, psychologists, therapists and religious/spiritual groups. Many people rely upon a combination of different support networks.
Engagement: although staying involved in regular activities may require making adaptations or seeking alternative activities, the physical and emotional benefits make it worth the effort.*
Q: Is there such a thing as worker's Scleroderma?
A: 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. According to the guidelines, the following is persuasive evidence that a worker's scleroderma is due to the nature of the employment:
The scleroderma, as defined below, is diagnosed by a licensed internist or rheumatologist, and;
The worker was exposed to substantial levels of silica dust in the employment before the diagnosis of scleroderma is made.
The level of exposure to silica dust is considered "substantial" when it is continuous and long-term, or when it is of short duration but intense. Scleroderma has been observed among workers occupationally exposed to silica dust for cumulative periods ranging from as few as 3 years to as many as 43 years. Case reports indicate that workers diagnosed with scleroderma have been exposed to extremely high levels of silica dust. Such exposure is exemplified by mining or sandblasting in an enclosed space. Occupational settings and processes that may expose workers to substantial levels of silica dust include:
Manufacture of abrasives, grinding and scouring compounds, moulds for castings, fillers for paints and mastic, glass, optical equipment, pottery, ceramics, electronic components, fibreglass, radio and TV components
Brick and cement work
Buffing, metal polishing and grinding
Working in foundries (ferrous and non-ferrous)
Working in quarries
Working at steel plants
Stone and clay making*
Q. Should I feel so tired all the time?
A. Everybody gets tired from time to time. Fatigue from scleroderma, however, is different from normal tiredness because it is often unrelated to physical exertion and not improved by getting enough rest. For many people, fatigue from scleroderma is debilitating and impacts the ability to go about daily activities more than any other symptom of the disease. Of the more than 400 Canadians with scleroderma surveyed in the recent Canadian Scleroderma Research Group survey, almost 90% said that 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. Many factors contribute to the fatigue experienced by people who have scleroderma, and there is no easy remedy. There are some steps that can be taken to reduce the impact of fatigue. Light, consistent exercise can help keep the body strong and boost energy. Similarly, eating a nutritional diet and maintaining a healthy weight can help combat fatigue. Finding enough time to rest, pacing oneself through the course of the day, avoiding overexertion and managing stress are all critical to easing fatigue.*
Q: Does Scleroderma cause depression?
A. It is important to distinguish clinical depression from the sadness and other emotional distress that often go along with living with a disease like scleroderma. Clinical depression is characterized by a low or sad mood most of the day, almost every day, or the loss of interest in all, or most activities, along with a number of other emotional and physical symptoms. 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% or so among people with diseases like scleroderma. When symptoms of depression are present that make it difficult to cope with living with scleroderma or any other aspect of daily life, it is a good idea to consult with a health care provider. 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. While these may or may not constitute clinical depression, people who experience significant distress may benefit from professional support. Others may find different kinds of support useful, such as from family, friends, or others living with scleroderma. Staying active, both physically and socially, is also helpful for many people.*
Q. I am having trouble sleeping is this normal?
A. It is estimated that approximately 30% of the general population complains of sleep problems with about 10% reporting problems that affect their ability to function. There has not been very much research on sleep in scleroderma, but there is reason to believe that sleep problems may be much more common among people with scleroderma when compared to the general population. A recent survey of more than 400 Canadians with scleroderma found that 76% of those surveyed reported difficulty sleeping at least some of the time and 59% said that poor sleep affected their ability to function at least moderately. Many studies have found that older people and women, particularly in the postmenopausal years, have difficulty with sleep and that sleep can 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 can also be uncomfortable and painful, making sleep difficult. Given the importance of sleep, it is important to let your health care provider know if you are having any problems with your sleep.*
Q. What can I do to manage Raynaud's in the winter months?
A. Raynaud's phenomenon occurs when the blood vessels in the fingers and toes contract excessively following exposure to cold or as a result of anxiety. The resulting reduction in blood flow causes the extremities to become blue or white in colour. When severe, there is pain and numbness as well. Raynaud’s is brought on by a cold environment or stress. Avoiding the cold or providing protection from the cold should be priorities for managing Raynaud’s in the winter. Wearing multiple thin layers of clothing is more effective to wearing one bulky layer. Layers should not be too tight so they do not restrict circulation. Raynaud's can also be triggered by stress. Stress restricts blood flow and thus makes Raynaud's worse; working on reducing stress should be a goal for those suffering from Raynaud's.
Q. How do I treat and prevent digital ulcerations?
A. Digital ulcers are skin sores that most often occur on the fingers, but can also occur on toes and joints. These ulcers are extremely painful and difficult to heal. They occur because there is limited circulation to these areas. Ulcers often start as a cut that doesn’t get enough blood to heal properly. Preventing and healing ulcers can be difficult, however strategies that have proven effective include keeping the hands protected and ensuring adequate blood flow to the fingers. Protective measures include wearing protective gloves when doing manually intensive labour, keeping skin moisturized with non-scented lotions, keeping ulcers moisturized to assist in healing, and keeping hands warm. Tips to keep blood flowing to the fingers include wearing loose clothing, preventing friction or pressure on the ulcer, and massaging the fingers and exercising the hands.
Q. Why do people with scleroderma get heartburn and difficulty swallowing?
A. Heartburn is caused by the dysfunction of the valve that separates the stomach and the esophagus, which allows stomach acid to flow backwards up into the esophagus thereby causing heartburn. Abnormal contractions of the esophagus can also occur, which prevent acid from being cleared properly leading to difficulty swallowing. Strategies for coping with heartburn include using over the counter medications that neutralize or reduce stomach acids, elevating the head of the bed, eating smaller meals and avoiding foods that cause heartburn. Chewing food well, eating slowly, and drinking fluids with meals can also help with heartburn.
Q. Why should I be monitoring my blood pressure regularly if I have systemic Scleroderma?
A. Regular self-monitoring of blood pressure is crucial in newly diagnosed individuals. Increases in blood pressure signify the onset of hypertension. Hypertension requires rapid action by medical professionals as it may indicate renal crisis, which is a medical emergency and considered one of the most life threatening complication of Scleroderma. Renal crisis is the onset of high blood pressure, kidney failure and increased levels of urea and renin in the blood. Those individuals newly diagnosed with diffuse scleroderma are at a much higher risk of developing renal crisis compared to those with limited scleroderma. Rapid diagnosis and therapy are key to overcoming renal crisis.
Q. Why do my hands hurt and feel stiff? What can I do?
A. 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 can also be effective in combating stiffness.
Q. My mouth is so dry what can I do?
A: Dry mouth, also called Sjogren’s Syndrome, can be a troublesome symptom for those affected. Staying hydrated by frequently sipping beverages, sucking on hard candy or chewing gum may all help. Avoiding foods that dehydrate the mouth and body such as alcohol, caffeine or salty foods can prevent further dryness. Sometimes the mouth is so dry that it becomes difficult to swallow, in which case drinking liquids while eating may also help.
Q. Will smoking make my scleroderma symptoms worse?
A. Smoking is not a healthy choice for anyone. For people living with scleroderma smoking can have significant adverse effects on the the respiratory, vascular and gastrointestinal tracts. Lung problems can be very severe in scleroderma patients and smoking drastically complicates these problems. Smoking constricts blood vessels and can increase the severity of Raynauds symptoms. Smoking also makes the mouth dry which can worsen the symptoms of Sjorgens syndrome. Stop smoking to improve symptoms, quality of life and longevity.
Q: Is erectile dysfunction related to scleroderma?
A: Males living with scleroderma suffer more frequently from erectile dysfunction compared to males in the general population. Treatment can be a challenge as there is limited research. Doctors are likely to begin by ruling out any other potential causes of erectile dysfunction.