Autoimmunity in scleroderma: the origin, pathogenetic role, and clinical significance of autoantibodies

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Abstract
Systemic sclerosis, or scleroderma, is a systemic autoimmune disease manifest by vascular damage and fibrosis within the skin and visceral organs. Whereas scleroderma is a heterogenous disorder in terms of disease symptoms and clinical course, scleroderma–specific autoantibody profiles associate strongly with distinct clinical phenotypes, making serologic testing of great diagnostic aid. This review will focus on the clinical significance and the potential pathogenic role of autoantibodies in scleroderma. Novel autoantibody and phenotype associations discovered within the past year underscore the clinical utility of systemic sclerosis–associated autoantibodies. Whereas autoantibodies are generally believed to indicate the presence of ongoing tissue damage, some research suggests that the humoral immune response may play a role in generating such damage. Improvements in multiplex autoantibody assays will aid in the diagnosis and prognosis of the complications associated with systemic sclerosis. Continued research into autoantibody/phenotype associations could also yield critical insights into the pathogenesis of, and suggest novel therapeutic targets for, this chronic, debilitating disease.