特发性炎症性皮肌病中自身抗体的研究进展

Autoantibodies in idiopathic inflammatory dermatomyopathies

特发性炎症性皮肌病，包括皮肌病、临床无肌病性皮肌炎和多发性肌炎。不同类型的特发性炎症性皮肌病可累及不同靶器官如皮肤、肺和肌肉等，临床表现比较复杂。肌炎特异性自身抗体和肌炎相关性自身抗体在特发性炎症性皮肌病中阳性率较高，与临床特征密切相关，在诊断中更具有特异性。部分患者血清中还存在一些与肌炎发生关系不密切的自身抗体，如抗临床无肌病性皮肌炎-140抗体、抗小泛素样修饰酶抗体，因此不应称之为肌炎特异性自身抗体，而应单独列为特发性炎症性皮肌病的特异性抗体。

Idiopathic inflammatory dermatomyopathies have been historically defined by broad clinical and pathological criteria, whose spectrum of clinical illness includes clinically amyopathic dermatomyositis, classic dermatomyositis and polymyositis. Clinical manifestations of this entity are diverse with various degrees of muscular, cutaneous and pulmonary involvement. Myositis-specific autoantibodies and myositis-associated autoantibodies are frequently detected in sera of patients with idiopathic inflammatory dermatomyopathies, some of which are closely associated with the clinical characteristics and specific for the diagnosis of idiopathic inflammatory dermatomyopathies. Recently, some autoantibodies such as anti-clinically amyopathic dermatomyositis （CADM） 140 antibody and anti-small ubiquitin-like modifier activating enzyme （SAE） antibody have been detected in sera of patients with idiopathic inflammatory dermatomyopathies, which are not closely associated with the initiation of myositis and should not be classified as myositis-specific autoantibodies, but as an independent subset of antibodies specific for idiopathic inflammatory dermatomyopathies.