重症幼年型皮肌炎的诊断、治疗进展

Diagnostic and therapeutic advances on severe juvenile dermatomyositis

重症幼年型皮肌炎除皮肤、肌肉损害外，还可出现全身多个系统和器官损害，直接威胁患儿的生命。影像学检查和特异性抗体检测技术的发展，有利于疾病的早期诊断。早期诊断、早期联合持续用药有助于改善预后。当前重症幼年型皮肌炎的治疗仍以大剂量糖皮质激素为主，静脉注射免疫球蛋白是一种快速有效的治疗方法，并可作为一线用药，6个月内可重复用药。对合并器官损害患儿可选择性使用环孢素、霉酚酸酯、他克莫司等免疫抑制剂，甚至是生物制剂、血浆置换、干细胞移植等，但疗效有待于进一步验证。

In addition to skin and muscle lesions, severe juvenile dermatomyositis can also cause multiple systemic and organ damage, which directly threatens the life of children. The development of imaging and specific antibody detection techniques is beneficial to the early diagnosis of severe juvenile dermatomyositis. Early diagnosis and regular combination of continuous medication can improve its prognosis. At present, the treatment of severe juvenile clermatomyosiris is mainly based on large doses of glucocorticoid. Intravenous immunoglobulin is a rapid and effective treatment and can be used as a first - line drug. It can be repeated in half a year. For children with combined organ damage, selective use of cyclosporine, mycophenolate mofetil, tacrolimus and other immunosuppressive agents, even biological agents, plasma exchange, stem cell transplantation, etc. , but the efficacy needs to be further verified.