Skarlis Charalampos, Michalakeas Nikolaos, Gerochristou Maria, Raftopoulou Sylvia, Marketos Nikolaos, Boki Kyriaki, Vassilopoulos Dimitrios, Stratigos Alexandros Panayiotis, Boumpas Dimitrios, Mavragani Clio
Department of Physiology, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
First Department of Dermatology and Venereology, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Mediterr J Rheumatol. 2023 Jun 30;34(2):275-278. doi: 10.31138/mjr.34.2.275. eCollection 2023 Jun.
Idiopathic inflammatory myopathies (IIMs) are a group of heterogeneous autoimmune diseases with a prevalence of 20 cases per 100000 of population. Despite their diversity, IMMs are characterised by several common clinical features such as muscle inflammation, proximal muscle weakness, abnormal electromyography and/or muscle biopsy. Over the last years, it has been increasingly recognised that an array of autoantibodies known as myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinct clinical phenotypes and diverse prognosis. Although the exact underlying mechanism of IIMs is not fully understood, accumulating data suggest that the activation of type I interferon pathway plays a central role in disease development. Previous studies have reported the upregulation of type I interferon (IFN) induced genes in peripheral blood and muscle biopsies derived from myositis patients. Given the heterogeneity of inflammatory myopathies along with the central role of type I IFN pathway in disease pathogenesis, the aim of the current study is to elucidate the link between distinct clinical phenotypes of inflammatory myopathies with the presence of serum MSAs or MAAs, as well as with type I IFN activation.
特发性炎性肌病（IIMs）是一组异质性自身免疫性疾病，每10万人中患病率为20例。尽管它们具有多样性，但IIMs具有一些共同的临床特征，如肌肉炎症、近端肌无力、异常肌电图和/或肌肉活检。在过去几年中，人们越来越认识到，一系列被称为肌炎特异性抗体（MSAs）和肌炎相关抗体（MAAs）的自身抗体与不同的临床表型和多样的预后相关。尽管IIMs的确切潜在机制尚未完全了解，但越来越多的数据表明，I型干扰素途径的激活在疾病发展中起核心作用。先前的研究报道了肌炎患者外周血和肌肉活检中I型干扰素（IFN）诱导基因的上调。鉴于炎性肌病的异质性以及I型干扰素途径在疾病发病机制中的核心作用，本研究的目的是阐明炎性肌病不同临床表型与血清MSAs或MAAs的存在以及I型干扰素激活之间的联系。
