Univ Lyon, Université Claude Bernard Lyon 1, CNRS UMR-5310, INSERM U-1217, Institut NeuroMyoGène.
Département de Médecine Interne et Immunologie Clinique, Centre Hospitalo-Universitaire Edouard Herriot, Hospices Civils de Lyon, Lyon, France.
Curr Opin Rheumatol. 2019 Nov;31(6):634-642. doi: 10.1097/BOR.0000000000000653.
The present review describes the interferon (IFN)-signature currently emerging as a tool for the diagnosis of idiopathic inflammatory myopathies (IIMs), and aims at presenting the interests and limitations of this recent tool for the clinics and the research.
Recent in-vivo and in-vitro transcriptomic studies have evidenced the involvement of IFNs in the pathogenesis of IIMs. A correlation between the IFN-signature and the clinical severity of IIMs has been established. Moreover, studies pointed out differences in the IFN-signature regarding the IIM subgroup (dermatomyositis, polymyositis, inclusion body myositis, anti-synthetase syndrome, immuno-mediated necrotizing myopathies), raising the hypothesis of several pathogenic processes in IIMs.
IIM pathogenesis remains partially understood. IFN-signature represents one of the main recent advances in the field. IFN-signature was identified thanks to transcriptomic analyses of tissues or cells from IIM patients (muscle, skin, blood cells, muscle cells) and should allow to establish new diagnosis and better monitoring of IIM patients. It also provides a tool for investigation of IIM pathogenesis. Nevertheless, IFN-signature still requires accurate definition in order to standardize its use, notably in the clinical practice.
本综述描述了干扰素(IFN)特征目前作为特发性炎性肌病(IIM)诊断工具的出现,并旨在介绍这一最新工具在临床和研究中的应用的意义和局限性。
最近的体内和体外转录组学研究表明 IFNs 参与了 IIM 的发病机制。IFN 特征与 IIM 临床严重程度之间存在相关性已得到证实。此外,研究还指出了 IFN 特征在 IIM 亚组(皮肌炎、多发性肌炎、包涵体肌炎、抗合成酶综合征、免疫介导性坏死性肌病)之间的差异,提出了 IIM 中存在多种致病过程的假说。
IIM 的发病机制仍部分未知。IFN 特征是该领域的主要最新进展之一。IFN 特征是通过对来自 IIM 患者(肌肉、皮肤、血细胞、肌细胞)的组织或细胞进行转录组分析而确定的,它应该有助于确立新的诊断和更好地监测 IIM 患者。它还为研究 IIM 的发病机制提供了一种工具。然而,IFN 特征仍需要更准确地定义,以便标准化其使用,特别是在临床实践中。