Clinical and pathophysiological significance of myositis-specific and myositis-associated autoantibodies

摘要

Autoantibodies to various cellular constituents are detected in the sera of patients with idiopathic inflammatory myopathy (IIM). These autoantibodies are closely associated with characteristic clinical manifestations of the diseases. Therefore, autoantibodies give us much information in clinical diagnosis, classification, prediction of prognosis and choice of treatment in patients with MM. During the last decade, novel myositis-specific autoantibodies have been identified, such as anti-CADM-140, anti-p155, anti-NXP-2, anti-SAE and anti-200/100, all of which except anti-200/100 are dermatomyositis-specific autoantibodies. The anti-CADM-140 antibody is associated with clinically amyopathic dermatomyositis and acute progressive interstitial pneumonia, anti-p155 is identified in malignancy-associated myositis and anti-200/100 is associated with necrotizing myositis. These new autoantibodies are extremely important because it had been thought that autoantibodies were negative in such subgroups. Target autoantigens have also been identified and their pathogenic roles in MM have been suggested. Our increased understanding of the autoantigenic properties of these targeted proteins will help us to reveal the pathophysiology of MM or its complications, and may lead to therapeutic development.