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December 1989

Systemic Sclerosis: Scleroderma

Author Affiliations

edited by Malcolm I. V. Jayson and Carol M. Black, 350 pp, with illus, New York, NY: John Wiley & Sons Inc; 1988

Arch Dermatol. 1989;125(12):1721. doi:10.1001/archderm.1989.01670240123037

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Scleroderma, or systemic sclerosis, is a striking example of a connective tissue disease characterized by severe end-stage fibrosis. During the past decade, however, it has been shown that the pathophysiology of systemic sclerosis involves vascular changes, an inflammatory reaction involving T and B cells, and marked collagen deposition. In this book, the editors attempted to review recent advances in the pathophysiology, diagnosis, and treatment of this interesting disease. The first three chapters are dedicated to an up-date of the classification, epidemiology, and genetic factors in systemic sclerosis. This section is followed by a discussion of the role of humoral and cellular immunity with emphasis on the role of cytokines in the behavior of fibroblasts, endothelial cell injury, and regulation of collagen synthesis. A chapter is dedicated to experimental models in scleroderma, of which chronic graft-vs-host disease was the most interesting, since the other models represent examples of fibrosis or vascular

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