Question:
Thank you Ronni for such an insightful post. We truly appreciate it. I can’t wait to read the next post. Thank you. — Love and Hugs From Sherry Messick Surviving Scleroderma http://www.SclerodermaSupport.com "Get Up, Stand Up and Fight to survive because Scleroderma Kicks you when you are down" "We are all Beacons Of Light for Each Other " Oprah Winfrey Tomorrow is promised to no one. Learn to say ‘I love you’…. Any way you can! / ____/_________ //////\ Have a Bewitching Halloween!! /// Q Q /// U // ___/
Response:
The Tri-State Chapter of the Scleroderma Foundation held a seminar in New York City on Oct. 18th, featuring some of today’s leading scleroderma researchers and physicians: Thomas A. Medsger, Jr., M.D., The Gerald Rodnan Professor of Medicine, University of Pittsburgh School of Medicine; Harry Spiera, M.D., Clinical Professor of Medicine, Mt. Sinai Medical Center; Lee Shapiro, M.D., Clinical Assistant Professor of Medicine, Albany Medical College and Chief of Rheumatology, St. Peter’s Hospital; and Carol Artlett, Ph.D., Assistant Research Professor, Division of Rheumatology, Thomas Jefferson University in Philadelphia. The panelists provided an overview of scleroderma, spotlighting the most recent developments and "hot research topics." As promised, I am providing highlights from this well-planned seminar (thanks to Amy Burkett, SF Tri-State Chapter Administrator): Although the cause of scleroderma remains a mystery, panelists believe there have been major developments in treatments over the past 20 years, and are optimistic that current research will yield more exciting and possibly disease-altering results. Patients with kidney involvement, for example, have a much better prognosis today than in the 1970s, noted Dr. Shapiro. "Prior to 1970, scleroderma renal involvement always meant the patient would die," he said. With the advent of dialysis, in 1970, patients had a more optimistic future, but they were dependent on dialysis machines. In 1977, the development of captopril for renal hypertension was hailed as a true life-saver for renal hypertension. Today, the challenge is to predict when a renal crisis might occur for a patient and to get immediate treatment. Physicians still are unable to identify who is most at risk for kidney involvement, said Dr. Shapiro. Scleroderma may be difficult to understand because it is not one disease, said Dr. Medsger. "It may be a family of 10 or 20 different diseases," he said. However, he did outline three major categories of scleroderma: localized, limited/CREST and diffuse. Localized scleroderma may consist of morphea or linear scleroderma, eosinophilic fasciitis, or eosinophilia-myalgia syndromes caused by epidemic or environmental factors (such as outbreaks caused by tainted batches of tryptophan and a cooking oil from Spain). Although many people with localized scleroderma fear it may evolve into systemic disease, Dr. Medsger said it does not. "It is believed that people who have localized disease and then get systemic scleroderma actually have two different diseases." This, however, is rare. Systemic scleroderma shares characteristics with a spectrum of other connective tissue diseases, including vasculitis, rheumatoid arthritis, systemic lupus and polymyositis/dermatomyositis. Blood vessels are the common target. Immune mechanisms also are involved in the tissue injury of these diseases, but it is unclear whether or not the immune disorder is primary (causes the disease) or secondary (results from a trigger). Both CREST (also known as limited) and diffuse scleroderma are systemic diseases, stressed Dr. Medsger. The terms "limited" and "diffuse" are abbreviations for limited and diffuse cutaneous scleroderma, referring to the extent of skin involvement. People with diffuse SD generally have widespread and rapidly progressing skin involvement, usually going above the elbows and knees. Lung, heart or kidney problems also come in the early stage (if they do develop). With limited/CREST, there is restricted and non-progressive skin involvement. Organ involvement, most notably pulmonary and malabsorption problems, come later. Patients may also have overlap with the other connective tissue diseases, such as lupus, sharing those symptoms too. Dr. Medsger made additional comparisons of limited and diffuse SD: gastrointestinal LIMITED DIFFUSE esophageal involvement 75% 75% malabsorption 5% 2% pulmonary hypertension 5-10% <1% severe fibrosis 5% 15% heart 1% 5-10% kidney crisis <1% 15-20% About 80-85% of people with SSc (systemic sclerosis) have one of seven antibodies associated with the disease: SCL-70, anti-centromere, anti-RNA polymerase III, anti-Th, anti-U3RNP, anti-PM-Scl and anti-UIRNP. Both Drs. Medsger and Spiera are proponents of D-penicillamine as an effective drug in modifying scleroderma, when prescribed in the early stages of the disease. They stressed, however, that the drug is not for every case, and that it seems to work best for patients with the anti-RNA polymerase III antibody. On the question of whether or not scleroderma is inherited, Dr. Medsger said studies suggest that certain inherited genes may influence the formation of antibodies involved with the disease. The genes may stimulate proteins (cytokines) that are capable of activating cells involved with SSc. Another post will follow later describing the "hot topics of research."
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