Despite the fact that a cure has not yet been found for scleroderma, recent advances in research and treatment offer scleroderma patients improved life expectancy and the hope for longer and more productive lives. Research has resulted in improved symptom management, prevention of organ damage and an improvement in the quality of life for people living with scleroderma. In the past two decades, multidisciplinary teams have uncovered new clues to understanding the disease, which is an important step toward prevention and cure. Scientists at universities and medical centres throughout Canada, the United States and other parts of the world are actively conducting scleroderma research. Studies of the immune system, genetics, cell biology, and molecular biology have helped reveal the causes of scleroderma, improve existing treatment, and create entirely new treatment approaches.
Some advances in the understanding or treatment of scleroderma are highlighted below:
- Building on research that identified a gene associated with scleroderma in Oklahoma Choctaw Native Americans, scientists are using new technology to look for other genes associated with the disease’s development and severity.
- The drug cyclophosphamide has been found effective in treating lung fibrosis. One recent study suggested that treating lung problems early with this immunosuppressive drug may help prevent further damage and increase chances of survival. In further research assessing the impact of cyclophosphamide on quality of life in people with lung involvement, 47 percent of people on the drug reported their health was somewhat or much better after one year, compared with 18 percent in the placebo group.
- ACE inhibitors are used increasingly for scleroderma-related kidney problems. For the past two decades, ACE inhibitors have greatly reduced the risk of kidney failure in people with scleroderma. Now there is evidence that use of ACE inhibitors can actually heal the kidneys of people on dialysis for scleroderma related kidney failure. As many as half the people who continue to use ACE inhibitors while on dialysis may be able to go off dialysis in 12 to 18 months.
- Several drugs are now available to treat pulmonary hypertension. Previously, pulmonary hypertension was associated with poor outcomes, but medications such as prostacyclins, endothelin-receptor antagonists, and phosphodiesterase inhibitors—epoprostenol sodium, bosentan, and sildenafil—have increased the quality of life and life expectancy for people with this dangerous form of lung damage.
Other studies are examining the following:
- The identification of several early immune system targets in scleroderma. Building on this research, scientists are continuing to look for ways to treat scleroderma earlier, before it has a chance to cause irreparable damage.
- Researchers believe that although factors related to both genetics and socioeconomic status may play a role, autoantibodies may be the primary reason that African Americans often have severe cases of the disease. A current study is examining the theory that scleroderma is more aggressive and associated with more extensive organ damage and a worse prognosis in non-Caucasians. Researchers hope that by better understanding the factors involved in scleroderma, they can design interventions that would improve the course and outcome of the disease.
- By studying changes in the tiny blood vessels of scleroderma patients, scientists hope to find the cause of cold sensitivity in Raynaud’s phenomenon and a way to control the problem.
- Studies have shown that certain chemicals called cytokines, made from cells in the body, enhance the development of increased collagen. New agents that counteract these cytokines may be helpful in preventing skin thickening.
- Scientists hope to find clues about skin changes in scleroderma by studying laboratory mice. Research has shown that laboratory mice with a particular genetic defect suffer from skin changes. Similar to scleroderma, these changes are caused by an over accumulation of collagen, leading to thick skin and patchy hair loss. Scientists are also working to establish mouse models for other problems related to scleroderma. These models will make it easier to understand these problems and develop treatments for them.
Scleroderma research continues to advance as scientists and doctors learn more about the disease’s mechanism of action and development. The ultimate research goal is to translate findings into improved treatment, patient care and possibly even a cure.
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Scleroderma Association of Saskatchewan
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