Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
Department of Pediatric Pneumology, Immunology and Critical Care Medicine and SPZ (Center for Chronically Sick Children), Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.
RMD Open. 2022 Feb;8(1). doi: 10.1136/rmdopen-2021-001934.
To evaluate sialic acid binding Ig-like lectin 1 (SIGLEC1) expression on monocytes by flow cytometry as a type I interferon biomarker in idiopathic inflammatory myopathies (IIM).
We performed a retrospective analysis of adult and paediatric patients with the diagnosis of IIM. SIGLEC1 expression was assessed by flow cytometry and was compared with Physician Global Assessment or Childhood Myositis Assessment Scale disease activity scores. Mann Whitney U test and receiver operating characteristic curves were used for cross-sectional data analysis (n=96), two-level mixed-effects linear regression model for longitudinal analyses (n=26, 110 visits). Response to treatment was analysed in 14 patients within 12 months, using Wilcoxon test. SIGLEC1 was compared with interferon-stimulated gene 15/MxA status by immunohistochemical staining of muscle biopsies (n=17).
96 patients with adult (a) and juvenile (j) dermatomyositis (DM, n=38), antisynthetase syndrome (AS, n=19), immune-mediated necrotising myopathy (IMNM, n=8), inclusion body myositis (IBM, n=9) and overlap myositis (n=22) were included. SIGLEC1 distinguished significantly between active and inactive disease with an area under the curve of 0.92 (95% CI 0.83 to 1) in DM and correlated with disease activity longitudinally (aDM: standardised beta=0.54, p<0.001; jDM: standardised beta=-0.70, p<0.001). Response to treatment in DM was associated with a decreasing SIGLEC1 (p<0.01, Wilcoxon test). SIGLEC1 was found upregulated in 8 of 19 patients with AS, 2 of 9 patients with IBM but not in IMNM.
SIGLEC1 is a candidate biomarker to assess type I interferon activity in IIM and proved useful for monitoring disease activity and response to treatment in juvenile and adult DM.
通过流式细胞术评估唾液酸结合免疫球蛋白样凝集素 1（SIGLEC1）在特发性炎性肌病（IIM）中的单核细胞表达，作为 I 型干扰素的生物标志物。
我们对成人和儿科特发性炎性肌病患者进行了回顾性分析。通过流式细胞术评估 SIGLEC1 的表达，并与医师整体评估或儿童肌炎评估量表的疾病活动评分进行比较。采用 Mann-Whitney U 检验和受试者工作特征曲线进行横断面数据分析（n=96），采用两水平混合效应线性回归模型进行纵向分析（n=26，110 次就诊）。在 12 个月内对 14 例患者的治疗反应进行了分析，采用 Wilcoxon 检验。通过肌肉活检的免疫组织化学染色比较 SIGLEC1 与干扰素刺激基因 15/MxA 状态（n=17）。
纳入 96 例成人（a）和青少年（j）皮肌炎（DM，n=38）、抗合成酶综合征（AS，n=19）、免疫介导性坏死性肌病（IMNM，n=8）、包涵体肌炎（IBM，n=9）和重叠性肌炎（n=22）患者。SIGLEC1 在 DM 中能显著区分活动期和非活动期疾病，曲线下面积为 0.92（95%CI 0.83 至 1），并与疾病的纵向活动相关（aDM：标准化β=0.54，p<0.001；jDM：标准化β=-0.70，p<0.001）。DM 患者的治疗反应与 SIGLEC1 降低相关（p<0.01，Wilcoxon 检验）。AS 患者中有 8 例、IBM 患者中有 2 例 SIGLEC1 上调，但在 IMNM 患者中未见上调。
SIGLEC1 是评估 IIM 中 I 型干扰素活性的候选生物标志物，在青少年和成年 DM 中对监测疾病活动和治疗反应很有用。
