Department of Frontier Medicine, St. Marianna University School of Medicine, Kawasaki, Japan.
Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health (NIH), Bethesda, MD.
Rheumatology (Oxford). 2022 Jul 6;61(7):2969-2977. doi: 10.1093/rheumatology/keab846.
JDM is an inflammatory myopathy characterized by prominent vasculopathy. AECAs are frequently detected in inflammatory and autoimmune diseases. We sought to determine whether AECAs correlate with clinical features of JDM, and thus serve as biomarkers to guide therapy or predict outcome.
Plasma samples from 63 patients with JDM, 49 patients with polyarticular JIA and 40 juvenile healthy controls were used to detect anti-heat shock cognate 71 kDa protein (HSC70) autoantibodies, a newly identified AECA, in ELISA assays. Clinical features were compared between JDM patients with and without anti-HSC70 autoantibodies.
Anti-HSC70 autoantibodies were detected in 35% of patients with JDM, in 0% of patients with JIA (P < 0.0001) and in 0% of healthy donors (P < 0.0001). Both the presence of cutaneous ulcers (59% vs 17%, P < 0.002) and the use of wheelchairs and/or assistive devices (64% vs 27%, P < 0.007) were strongly associated with anti-HSC70 autoantibodies in JDM. High scores on the severity of myositis damage measures at the time of measurement of anti-HSC70 autoantibodies and an increased number of hospitalizations were also associated with anti-HSC70 autoantibodies. Intravenous immunoglobulin therapy was used more often in anti-HSC70 autoantibody-positive patients.
Anti-HCS70 autoantibodies are detected frequently in children with JDM and are novel myositis-associated autoantibodies correlating with disease severity.
JDM 是一种以显著血管病变为特征的炎症性肌病。AECA 常在炎症性和自身免疫性疾病中被检测到。我们旨在确定 AECA 是否与 JDM 的临床特征相关，从而作为指导治疗或预测预后的生物标志物。
使用 ELISA 检测来自 63 例 JDM 患者、49 例多关节 JIA 患者和 40 例青少年健康对照者的血浆样本，以检测新发现的抗热休克同源 71kDa 蛋白（HSC70）自身抗体。比较 JDM 患者有无抗-HSC70 自身抗体的临床特征。
在 35%的 JDM 患者中检测到抗-HSC70 自身抗体，在 0%的 JIA 患者中（P<0.0001）和 0%的健康供体中（P<0.0001）均未检测到。JDM 患者中存在皮肤溃疡（59%比 17%，P<0.002）和使用轮椅和/或辅助设备（64%比 27%，P<0.007）与抗-HSC70 自身抗体密切相关。在检测抗-HSC70 自身抗体时，肌炎损伤严重程度的评分较高和住院次数增加也与抗-HSC70 自身抗体相关。抗-HSC70 自身抗体阳性患者更常使用静脉注射免疫球蛋白治疗。
抗-HCS70 自身抗体在 JDM 患儿中经常被检测到，是与疾病严重程度相关的新型肌炎相关自身抗体。
