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pathology perspectives BY RUTH WERNER CREST SYNDROME CREST syndrome. Sounds like a term referring to dental hygiene, doesn't it? In fact, it is an acronym for a group of signs and symptoms that are associated with one form of scleroderma, an autoimmune disease. Scleroderma isn't particularly common; about 300,000 people in the United States have been diagnosed with it. But exploring this disease reveals some things about chronic inflammation, immune system dysfunction, and the nature of connective tissue that might intrigue massage therapists. WHAT IS SCLERODERMA? Scleroderma (literally, "hard skin") refers to a group of autoimmune diseases with a particular outcome: whatever the tissue attacked, the body responds by investing the nearby areas with deposits of collagen and other connective tissue extracellular matrix, thus causing the area to become destructively hardened or scarred. Most forms of scleroderma affect the skin distal to the elbows and knees, but it may also affect the skin of the trunk, face, or neck. Areas of involvement can be rounded or streaked. In some cases, these deposits of collagen can also affect the internal organs (especially the lungs, gastrointestinal tract, and kidneys), and the rarest form of the disease affects the organs only, with no skin involvement. The etiology of scleroderma is not at all well understood. Gender clearly has a role: it occurs in women about four times more frequently than in men. (Interestingly, the Choctaw women of Oklahoma have the highest incidence of scleroderma.) Most cases are diagnosed between ages 30 and 50. It seems to arise most often in families where certain other autoimmune diseases occur, specifically lupus, rheumatoid arthritis, and Hashimoto's thyroiditis. It seems clear that the combination of genetic susceptibility and environmental toxic exposures (silica, polyvinyl chloride, benzene, and trichloroethylene are at the top of the list) provide powerful triggers for the immune system to malfunction, but in most cases the exact causes of this disease are unknown.1 When scleroderma occurs, the immune system appears to attack the lining, or intima, of small- and medium-sized blood vessels. As part of the inflammatory response, other immune system cells collect in the affected areas: macrophages, eosinophils, basophils, antibody producing B cells, and mast cells (all associated with pro-inflammatory activity). These cells secrete pro- inflammatory chemicals, while regulator T cells, which would ordinarily suppress the inflammatory response, are conspicuously absent. The concentration and heightened activity of these white blood cells appear to be early indicators of the disease process. Even in situations without autoimmune dysfunction, it is readily seen that chronic inflammation leads to the development of excessive scar tissue. In scleroderma, that process seems to run completely amok. Fibroblasts (connective tissue producing cells) that live in the skin connect with your colleagues on massageprofessionals.com 107