Systemic sclerosis

Abstract

Systemic sclerosis is an autoimmune disease of unknown origin characterized by excessive deposition of collagen and other connective tissue macromolecules in skin and multiple internal organs, severe alterations in the microvasculature, and humoral and cellular immunological abnormalities. Although the most apparent and almost universal clinical features of systemic sclerosis are related to the progressive thickening and fibrosis of the skin, some degree of involvement of multiple internal organs is uniformly present even when not clinically apparent. This chapter will examine the clinical aspects and pathogenesis of the disease and review some promising therapies that have been and are being developed that might modify the natural course of this serious and often lethal disorder. © 2006 Humana Press Inc.