Advances in the classification and management of idiopathic inflammatory myopathies.

Autoantibodies can aid in the identification of disease subtypes and their associated severe complications, such as cancer or interstitial lung disease.

自身抗体有助于识别疾病亚型及其相关的严重并发症，例如癌症或间质性肺病。

Patients with idiopathic inflammatory myopathies need to be managed in a multidisciplinary setting.

特发性炎症性肌病

Treatment with intravenous immunoglobulins is efficacious in patients with refractory dermatomyositis, and can result in improvements in disease activity in the skin and muscle.

在多学科环境中进行管理

Numerous randomised controlled trials are underway testing potential therapeutic agents that hold promise for the treatment of idiopathic inflammatory myopathies.

正在进行多项随机对照试验，测试对特发性炎症性肌病治疗有希望的潜在治疗剂。

Other advances include the identification of pathophysiological mechanisms.

其他进展包括识别病理生理机制。

Induction of interferons in patients with dermatomyositis leads to the upregulation of interferon-stimulated genes in blood, skin, and muscle tissue.

在皮肌炎患者中诱导干扰素会导致血液、皮肤和肌肉组织中干扰素刺激基因的上调。

The interferon-induced transcripts could yield diagnostic biomarkers and biomarkers for monitoring disease activity.

干扰素诱导的转录物可能产生用于诊断的生物标志物以及监测疾病活动的生物标志物。

The identification of these potential biomarkers has also propelled the development of therapies targeting the interferon pathway-either upstream by using monoclonal autoantibodies or by blocking downstream signalling pathways via JAK inhibitors.

这些潜在生物标志物的识别也推动了针对干扰素通路的治疗开发——无论是通过使用单克隆自身抗体在上游进行干预，还是通过JAK抑制剂阻断下游信号通路。

A promising strategy for patients with refractory disease is targeting B cells with CD19-targeting chimeric antigen receptor T-cell therapy.

对于难治性疾病患者来说，一种有前景的策略是使用靶向CD19的嵌合抗原受体T细胞疗法针对B细胞。

Treatments targeting T cell lymphocytes and specific T-cell subsets are also under investigation.

目前也在研究针对T细胞淋巴细胞及其特定亚群的治疗方法。