Kamperman Renske G, Veldkamp Saskia R, Evers Sanne W, Lim Johan, van Schaik Ivo, van Royen-Kerkhof Annet, van Wijk Femke, van der Kooi Anneke J, Jansen Marc, Raaphorst Joost
Department of Neurology, Amsterdam University Medical Centre, Location AMC, Amsterdam, The Netherlands.
Center for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
Rheumatology (Oxford). 2025 May 1;64(5):2979-2986. doi: 10.1093/rheumatology/keae630.
Novel biomarkers are needed to guide therapy in idiopathic inflammatory myopathies (IIM). Expression of Siglec-1, a type I interferon biomarker, was examined in adult patients with IIM in relation to disease activity and treatment response.
We analyzed PBMC samples from 19 newly diagnosed adult IIM patients who participated in a phase-2 pilot study on efficacy of intravenous immunoglobulin (IVIG) monotherapy, and from 9 healthy controls. Siglec-1 expression on monocytes was measured by flow cytometry before and after treatment, and was evaluated in relation to IIM subtype, physician global activity (PhGA) scores, manual muscle strength (MMT) and the total improvement score (TIS).
Diagnoses included dermatomyositis (DM; n = 9), immune-mediated necrotizing myopathy (IMNM; n = 5), non-specific/overlap myositis (NSM/OM; n = 4) and antisynthetase syndrome (ASyS; n = 1). All patients showed increased Siglec-1 expression at baseline. Relative median fluorescence intensity of Siglec-1 was highest in patients with DM. After 9 weeks, follow-up samples were available for 15 patients of whom 10 patients showed a decline in Siglec-1 expression. In DM, Siglec-1 correlated with disease activity (MMT; rs = -0.603, P = 0.013 and PhGA; rs = 0.783, P < 0.001) and with the TIS (rs = -0.786, P = 0.036).
Siglec-1 was increased in treatment-naive IIM patients and showed a decline after IVIG monotherapy. In DM, Siglec-1 expression correlated with relevant clinical measures. This underlines the dynamic role of type I IFN in IIM and the biomarker potential of Siglec-1, in particular in DM. These findings should be further validated in larger cohorts with longer follow-up.
需要新型生物标志物来指导特发性炎性肌病（IIM）的治疗。研究了I型干扰素生物标志物Siglec-1在成年IIM患者中的表达与疾病活动度和治疗反应的关系。
我们分析了19名参加静脉注射免疫球蛋白（IVIG）单药治疗疗效2期试点研究的新诊断成年IIM患者以及9名健康对照者的外周血单个核细胞（PBMC）样本。通过流式细胞术在治疗前后测量单核细胞上Siglec-1的表达，并根据IIM亚型、医生整体活动度（PhGA）评分、手动肌力（MMT）和总改善评分（TIS）进行评估。
诊断包括皮肌炎（DM；n = 9）、免疫介导的坏死性肌病（IMNM；n = 5）、非特异性/重叠性肌炎（NSM/OM；n = 4）和抗合成酶综合征（ASyS；n = 1）。所有患者在基线时Siglec-1表达均升高。Siglec-1的相对中位荧光强度在DM患者中最高。9周后，15名患者有随访样本，其中10名患者Siglec-1表达下降。在DM中，Siglec-1与疾病活动度（MMT；rs = -0.603，P = 0.013和PhGA；rs = 0.783，P < 0.001）以及TIS（rs = -0.786，P = 0.036）相关。
初治IIM患者中Siglec-1升高，IVIG单药治疗后下降。在DM中，Siglec-1表达与相关临床指标相关。这突出了I型干扰素在IIM中的动态作用以及Siglec-1的生物标志物潜力，尤其是在DM中。这些发现应在更大队列、更长随访时间的研究中进一步验证。
