Immunopathogenesis of scleroderma--evolving concepts

Abstract

Scleroderma, or systemic sclerosis, is a connective tissue disease which may affect various organ systems including skin, lungs, gastrointestinal tract, cardiovascular system and kidneys. While the etiology is not clear, it is currently believed that scleroderma may represent an autoimmune response to an unknown antigen. In this regard, there is evidence that both humoral and cellular immunity may play roles. The pathophysiology is complex and consists of three major features: (1) vascular damage; (2) mononuclear cellular infiltrates; and (3) massive deposition of newly synthesized connective tissue, mainly collagen. The major pathologic features of scleroderma and the roles of humoral and cellular immunity in its pathogenesis are reviewed and summarized.