Pathophysiological and clinical significance of anti-nuclear antibodies in systemic sclerosis

Abstract

Systemic sclerosis (M34.0) is a polysyndromic autoimmune disease characterized by progressive fibrosis of the skin, musculoskeletal system, internal organs (lungs, kidneys, heart, digestive system) and vasospastic disturbances.It occurs in around 30 people per million population per year and there are an estimated 125,000 active cases in the United States and perhaps 2.5 million worldwide. Although systemic sclerosis is infrequent, it has a high morbidity and mortality and serious life threatening complications. The main marker of an abnormal immune response is circulating autoantibodies, which are found in more than 95% of patients with systemic sclerosis. It is important to study the clinical, immunological, diagnostic and prognostic significance of autoantibodies.