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Rheumatology team helps lead lung study by Mary Helen Yarborough Public Relations A team of MUSC scleroderma experts have completed a six-year study that has resulted in hope for thousands afflicted with scleroderma-related lung disease.   The results of the Scleroderma Lung Study (SLS) will be published June 22 in the New England Journal of Medicine, which also has been co-authored by the MUSC Department of Medicine researchers: Richard Silver, M.D., Marcy Bolster, M.D., Edwin Smith, M.D. (from the Division of Rheumatology and Immunology), and Charlie Strange, M.D. (from the Division of Pulmonary and Critical Care Medicine).   Scleroderma is an autoimmune disease affecting  connective tissue, primarily the skin, but also internal organs, including the lungs.   MUSC was among 13 U.S. clinical centers that participated in a study of the effectiveness of the drug cyclophosphamide in treating scleroderma lung disease.   The double-blind, placebo-controlled study provided “the best clinical trial design we have,” said Silver, director of MUSC’s Division of Rheumatology and Immunology, who headed MUSC’s involvement in the $8-million study. While cyclophosphamide is no cure for the lung fibrosis that afflicts approximately half of the 300,000 scleroderma patients in the U.S., the study’s results show more promise than any other in the treatment of the disease, he explained.   Silver said that those who took the drug, versus those who took a placebo, experienced a 3-percent lung function improvement. While that may seem slight, Silver said that this and other improvements were significant.   “Of additional importance are other treatment outcomes,” Silver explained. “We saw an improvement in skin tightness, quality of life, and an improvement in the degree of shortness of breath.”   The SLS was coordinated at the University of California at Los Angeles (UCLA). MUSC served as one of the patient enrollment sites and also was one of the core sites. MUSC played a key role by providing analysis of all of the bronchoscopies of the participants, Silver said.   MUSC is noted for its long history of scleroderma research, and is one of the world’s top research and treatment facilities for the disease. Ongoing clinical and basic research on scleroderma is supported by  $1.5 million in grants from the National Institutes of Health (NIH), Scleroderma Foundation and Arthritis Foundation. The SLS study was funded by a grant from NIH’s National Heart, Lung and Blood Institute. What is scleroderma? Scleroderma is diagnosed as the result of an unusual thickening or swelling of the skin, especially on the hands and extending up the arms, as well as dilated blood vessels in the face, hands, nail folds and elsewhere. Some patients develop cutaneous deposits of calcium, and characteristic involvement of other organs including the lungs, muscle inflammation, and the kidneys.   The several types of scleroderma and related diseases with complications range from minor to life-threatening. The two broad categories are: localized scleroderma, which indicates distinct skin lesions, and systemic sclerosis, which indicates more uniform skin involvement and the potential for internal organ disease.   Meanwhile, scleroderma can involve almost every organ system including the skin, vascular system, lungs, gastrointestinal tract, heart, joints, muscles, etc. Further, this disease varies greatly from patient to patient and can dramatically impact someone’s life. Patients should receive care from a specialist (and often a team of specialists) with expertise in the management of scleroderma. Patients also benefit from support from family and friends, and learn to live with the disease to maintain a good quality of life. June is Scleroderma Awareness Month Scleroderma Awareness Month has prompted a number of national and statewide awareness-raising events that included Gov. Mark Sanford issuing a proclamation urging citizens to participate and seek continued research.   The S.C. chapter has various fund-raisers throughout the year to support the programs of the chapter and research.   In Charleston, fund-raisers include a make-believe tea, an annual golf tournament, bowl-a-thons, and raffles (for more information, contact Amy Parrish at (866) 557-3729, or sclerodermasc@hotmail.com). Statewide activities On June 17, a Stepping Out to Cure Scleroderma Walk will be held at Clemson University around the popular botanical gardens.   During the month of June, the S.C. chapter hosts several billboards promoting awareness of scleroderma in the Charleston area, schedules television and radio interviews, and publishes several newspaper articles. The South Carolina Chapter (formerly known as the Palmetto Chapter) won the Public Awareness Award in 1999 and 2000.   The South Carolina Chapter provides a safe place for patients, families and friends of those affected by scleroderma, and works closely with the national foundation to provide up-to-date information about the disease. The chapter has recently expanded its services to include the Columbia and Greenville/Spartanburg area.     Friday, June 16, 2006 Catalyst Online is published weekly, updated as needed and improved from time to time by the MUSC Office of Public Relations for the faculty, employees and students of the Medical University of South Carolina. Catalyst Online editor, Kim Draughn, can be reached at 792-4107 or by email, catalyst@musc.edu. Editorial copy can be submitted to Catalyst Online and to The Catalyst in print by fax, 792-6723, or by email to catalyst@musc.edu. To place an ad in The Catalyst hardcopy, call Island Publication at 849-1778, ext. 201.