抗合成酶综合征
皮肌炎
肌炎
医学
多发性肌炎
免疫学
炎性肌病
病理
间质性肺病
包涵体肌炎
自身抗体
结缔组织病
疾病
炎症
炎症反应
自身免疫性疾病
骨骼肌
肌肉疾病
全身性疾病
肌营养不良
作者
Y Allenbach,Olivier Benveniste
标识
DOI:10.1056/nejmra2415426
摘要
Inflammatory myopathies are a heterogeneous group of autoimmune diseases characterized by immune-mediated damage to skeletal muscle. They are classified into five major subtypes: inclusion-body myositis, immune-mediated necrotizing myopathies, antisynthetase syndrome, overlapping myositis, and dermatomyositis, each with distinct clinical features and outcomes. Inclusion-body myositis and immune-mediated necrotizing myopathies primarily affect muscle, with prognosis largely determined by functional impairment, whereas antisynthetase syndrome, overlapping myositis, and dermatomyositis are systemic diseases that can involve the skin, joints, and lungs and may be life-threatening. The majority of inflammatory myopathies are associated with myositis-specific autoantibodies, which inform diagnosis, subtype classification, and prognosis. Advances in understanding the distinct pathomechanisms underlying each subgroup now enable increasingly targeted therapeutic approaches.
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