Currently Funded Projects:
Dr. Sindhu Johnson
Thank you to the Scleroderma Society of Ontario and your donors for your ongoing support of Scleroderma research at the University of Toronto. Your support allows scleroderma research to be done here in Ontario. I am happy to share with you some highlights from recently published research.
Classification Criteria for Scleroderma Subsets.
Scleroderma can affect people in different ways. This has lead to grouping of different types of scleroderma using terms like “CREST syndrome,” “limited,” “diffuse,” “scleroderma sine scleroderma.” We found 14 different classification systems. We found that the classification system of Dr. LeRoy which used the terms “limited” and “diffuse” depending if the skin thickening extends beyond the elbow to be the most useful. It is easy to use and can help predict a patient’s outcome in the future.
Prognostic factors for survival in scleroderma associated pulmonary hypertension.
Pulmonary hypertension (high pressure in the blood vessels of the lungs) is the leading cause of death in Canadian scleroderma patients. We wanted to find patients characteristics which would predict survival. Our research found that certain genes (HLA DRw6, HLA DRw52) are associated with poor survival. Age, sex, and some scleroderma antibodies may be associated with survival. Lower pressures in the pulmonary artery and the right side of the heart are associated with better survival.
We currently are involved in a 3-phase study involving a retrospective cohort study, a belief elicitation study and a decision analysis looking at the effect of warfarin for improving survival in scleroderma associated pulmonary hypertension.
Your generosity has made all this research possible. Thank you for your support.
Sindhu Johnson MD FRCPC
Division of Rheumatology
University of Toronto
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University of Toronto Scleroderma Clinic
Sindu Johnson MD, FRCPC
I would like to express my sincere gratitude for your generous financial support. As a result, you are ensuring ongoing training of scleroderma research scientists for Canada. I would like to highlight some of the recent research findings your support has helped generate.
Quality of Life and Disability
We studied quality of life and disability in people with scleroderma compared to people with other rheumatic diseases. We foumd that people with scleroderma and arthritis suffer from more disability than prople with psoriatic arthritis, and suffer more pain than people with rheumatoid arthritis. Patients with more skin involvement and gastro-intestinal involvement tend to have more disability. Despite their pain and disability, the quality of life enjoyed by people with scleroderma was similar to people with rheumatoid arthritis, psoriatic arthritis and lupus.
Access To Care
Through a national survey of patients with scleroderma we evaluated use of the health care system. The average time to diagnosis over the last 3 decades was 2.4 years. Three quarters of Canadian scleroderma patients developed scleroderma between the ages of 30 and 59, the prime of their working life. Eight percent reported a blood relative with scleroderma. At diagnosis, less than half of patients had an electrocardiogram, echocardiogram, gastroscopy, or CT scan of the lungs. Over half of the patients had a chest x-ray and pulmonary function tests. Many patients may not need these test, but we feel that scleroderma patients should be educated about the disease so that they may discuss with their physician the need for specialized tests.
Pulmonary Veno-occlusive Disease in Scleroderma
In 2006, we were among the first centers to document pulmonary veno-occlusive disease as a rare cause of scleroderma associated pulmonary hypertension. We found that the need for treatment with oxygen, fluid collecting in the bottom of the lungs, and specific features on the CT scan of the lungs are suggestive of this rare condition.
Through this body of research we are gaining a better understanding of scleroderma in Canada-how the disease can afect the body, how the disease affects people's lives, and evaluating access to necessary health care.
Our current and up-coming research will evaluate both older and novel therapies. We are conducting a series of studies looking at improving survival in scleroderma associated pulmonary hypertension. We are also evaluating the effect of osteopathy, a form of complementary treatment.
This is a sample of the work that has resulted from your generous support. Your continued support will move scleroderma research forward in Canada. Together we can find the answers.
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Dr. Andrew Leask
Dr. Andrew Leask is a basic science researcher at the University of Western Ontario. He has just recently been approved for Scleroderma Society funding as a New Investigator. This five year award provides partial salary support for Dr. Leask in order to protect a significant amount of his time to carry out his research.
Dr. Leask is interested in pursuing the effects of fibrotic diseases, such as Scleroderma. It is known that fibrosis results in failure of organ function and can affect the kidney , liver, lung, skin and blood vessels. In Scleroderma, this is fatal, affecting all of these organs. Why fibrosis occurs is unknown. Therefore, there are no fibrotic therapies. His work has discovered a potential mediator of fibrosis which is over expressed in fibrotic tissues. The objective of this proposal is to learn what this mediator does and whether it is a good candidate against which to develop anti-fibrotic therapies.
Monies generated by the Scleroderma Society of Ontario have effectively funded the first year of a five year grant.
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Dr. Murray Baron
Dr. Murray Baron, The funds will be used to increase the capacity to perform scleroderma research in Canada. Mentors will be provided to young researchers in specific fields at multiple sites. This community of new researchers will complete their training and continue to interact, which will ensure cross-fertilization of ideas from one discipline to another. They will acquire the expertise to run independent research programs on multiple aspects of scleroderma, in both clinical and laboratory settings, and will become part of an expanded CSRG.
The CSRG was formed in 2004. This highly respected consortium of researchers and rheumatologists, at multiple sites in Canada has an established commitment to integrated, trans-disciplinary research. Its rheumatologists see scleroderma patients at 15 sites, and then enter detailed data into a central database that already includes one of the largest such cohorts in the world. Expansion to include the new trainees will increase its capability. Scleroderma patients will undoubtedly benefit from their work, but so will those coping with other Systemic Autoimmune Rheumatic Diseases [SARD], such as lupus and Sjogren's Syndrome patients.
