Plotz P H, Rider L G, Targoff I N, Raben N, O'Hanlon T P, Miller F W
National Institutes of Health, Bethesda, MD 20892, USA.
Ann Intern Med. 1995 May 1;122(9):715-24. doi: 10.7326/0003-4819-122-9-199505010-00010.
The myositis syndromes, the most common forms of which are polymyositis and dermatomyositis, are defined by idiopathic chronic inflammation in skeletal muscle. Although initially described more than a century ago, these diseases are so rare and heterogeneous that we have only a limited understanding of their causes and treatment. Recently, autoimmune responses to nuclear and cytoplasmic autoantigens that are unique to patients with myositis, the myositis-specific autoantibodies, have proved clinically useful in helping predict signs and symptoms of myositis, immunogenetics, responses to therapy, and prognosis. We summarize this new information on the variety and nature of these autoantibodies, their target epitopes, and their possible use in identifying causes, pathogenetic mechanisms, and better therapies for these increasingly recognized disorders.
肌炎综合征，最常见的形式是多发性肌炎和皮肌炎，其定义为骨骼肌的特发性慢性炎症。尽管这些疾病在一个多世纪前就首次被描述，但它们非常罕见且具有异质性，以至于我们对其病因和治疗的了解有限。最近，针对肌炎患者所特有的核和细胞质自身抗原的自身免疫反应，即肌炎特异性自身抗体，已被证明在帮助预测肌炎的体征和症状、免疫遗传学、治疗反应及预后方面具有临床实用性。我们总结了有关这些自身抗体的种类和性质、其靶表位以及它们在确定这些日益被认识的疾病的病因、发病机制和更好治疗方法方面可能用途的新信息。
