Hussain Altaf, Rawat Amit, Jindal Ankur Kumar, Gupta Anju, Singh Surjit
Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India.
Rheumatol Int. 2017 May;37(5):807-812. doi: 10.1007/s00296-017-3707-4. Epub 2017 Mar 22.
The objective of this study is to determine autoantibody profile in children with juvenile dermatomyositis (JDM). Children who were diagnosed with JDM (either recently diagnosed during the study period or follow-up patients) were included in the study. Autoantibodies were detected with commercially available Immunodot kit. Thirty patients were included in the study. Nine out of thirty patients (30%) were positive for one of the 12 autoantibodies tested. Anti-SRP antibody was most common antibody detected in 3 patients followed by anti-MDA-5 antibody in 2 patients; while anti-Jo1 antibody, anti-TIF1-γ antibody, anti-Mi-2 antibody, and anti-PM-Scl antibody were positive in 1 patient each. A different disease phenotype was observed with each autoantibody. The patient with anti-Jo1 antibody had a severe systemic disease in the form of interstitial lung disease; patients with anti-MDA-5 antibody and anti-Mi2 antibody had more severe skin disease with mild muscle disease and patients with anti-SRP antibody had significant skin and muscle disease. Anti-TIF1-γ and anti-PM-Scl antibodies were seen in patients with features of overlap syndrome (myositis-scleroderma). Estimation of autoantibodies may serve as an adjunct tool in delineating and defining distinct clinical phenotypes in children diagnosed with juvenile dermatomyositis. They may also help in prognostication.
本研究的目的是确定青少年皮肌炎（JDM）患儿的自身抗体谱。被诊断为JDM的儿童（在研究期间新近诊断或随访患者）纳入本研究。使用市售免疫斑点试剂盒检测自身抗体。30例患者纳入本研究。30例患者中有9例（30%）对所检测的12种自身抗体中的一种呈阳性。抗SRP抗体是最常见的检测到的抗体，3例患者呈阳性，其次是抗MDA-5抗体，2例患者呈阳性；而抗Jo1抗体、抗TIF1-γ抗体、抗Mi-2抗体和抗PM-Scl抗体各有1例患者呈阳性。每种自身抗体观察到不同的疾病表型。抗Jo1抗体阳性的患者患有以间质性肺病形式出现的严重全身性疾病；抗MDA-5抗体和抗Mi2抗体阳性的患者有更严重的皮肤病伴轻度肌肉病，抗SRP抗体阳性的患者有明显的皮肤和肌肉病。抗TIF1-γ和抗PM-Scl抗体见于有重叠综合征（肌炎-硬皮病）特征的患者。自身抗体的评估可作为一种辅助工具，用于描述和定义诊断为青少年皮肌炎的儿童的不同临床表型。它们也可能有助于预后判断。
