Differential Clinical Associations of Anti-Nuclear Matrix Protein 2 Autoantibodies in Patients With Idiopathic Inflammatory Myopathies.
Differential Clinical Associations of Anti-Nuclear Matrix Protein 2 Autoantibodies in Patients With Idiopathic Inflammatory Myopathies.
复制标题
特发性炎症性肌病患者抗核基质蛋白 2 自身抗体的差异临床关联。
DOI：
10.1002/art.40491
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发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Wang Guochun
中科院分区：
文献类型：
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作者：
Yang Hanbo;Lu Xin;Peng Qinglin;Jiang Wei;Shi Jingli;Zhang Yamei;Chen He;Wang Guochun
目的探讨特发性炎性肌病（IIMs）患者抗核基质蛋白2（NXP-2）自身抗体水平与疾病活动性以及钙质沉着严重程度之间的相关性。方法检测709例IIMs患者血清中抗NXP-2自身抗体水平，并采用MORC 3重组蛋白进行酶联免疫吸附测定。将抗恩智浦-2自身抗体患者分为2个亚组：伴和不伴钙质沉着的患者。抗恩智浦-2自身抗体水平与器官特异性疾病活动的相关性在横断面和纵向分析中研究了NXP-2自身抗体阳性的56例IIM患者的横断面分析（使用10 cm视觉模拟量表[VAS]评分）、血清肌酸激酶（CK）水平和钙质沉着严重程度。（38例无钙质沉着，18例有钙质沉着）显示，在无钙质沉着的患者中，抗-恩智浦-2自身抗体与医生对疾病活动性的总体评估、肌肉VAS评分和血清CK水平呈正相关，而在钙质沉着症患者中没有发现这种关联。纵向研究的结果显示，在无钙质沉着的患者中，抗NXP-2抗体水平与医生的总体评估以及全身、皮肤、胃肠道和肌肉VAS评分和血清CK水平之间存在强相关性，但在钙质沉着患者中，抗NXP-2抗体水平与医生的总体VAS和全身VAS评分之间仅存在中度相关性。值得注意的是，在无钙质沉着的患者中，抗恩智浦-2自身抗体在临床缓解期消失，但在疾病复发时再次出现。抗恩智浦-2抗体水平和钙质沉着症的严重程度之间没有关联observed.ConclusionThese研究结果表明，抗恩智浦-2自身抗体作为一个有用的标志物与IIM患者的疾病活动，特别是在没有钙质沉着症。在抗恩智浦-2自身抗体水平和疾病活动性之间观察到的差异相关性表明，有和没有钙质沉着症的患者之间可能存在表型差异。
ObjectiveTo investigate the associations between anti–nuclear matrix protein 2 (anti–NXP‐2) autoantibody levels and disease activity as well as calcinosis severity in patients with idiopathic inflammatory myopathies (IIMs).MethodsSerum levels of anti–NXP‐2 autoantibodies were determined in 709 patients with IIMs and also serially measured in the patients’ sera with an in‐house enzyme‐linked immunosorbent assay using MORC3 recombinant protein. Patients with anti–NXP‐2 autoantibodies were divided into 2 subgroups: those with and those without calcinosis. Associations of anti–NXP‐2 autoantibody levels with organ‐specific disease activity (using 10‐cm visual analog scale [VAS] scores), serum creatine kinase (CK) levels, and calcinosis severity were investigated in cross‐sectional and longitudinal analyses.ResultsA cross‐sectional analysis of 56 IIM patients with anti–NXP‐2 autoantibodies (38 without calcinosis and 18 with calcinosis) showed that in patients without calcinosis, the levels of anti–NXP‐2 autoantibodies were positively correlated with the physician's global assessment of disease activity and muscle VAS scores and serum CK levels, whereas no such association was found in patients with calcinosis. Results of the longitudinal study revealed strong correlations of anti–NXP‐2 antibody levels with the physician's global assessment and constitutional, cutaneous, gastrointestinal, and muscle VAS scores and serum CK levels in patients without calcinosis, but in patients with calcinosis, only a moderate correlation was observed between anti–NXP‐2 antibody levels and the physician's global VAS and constitutional VAS scores. Of note, in patients without calcinosis, anti–NXP‐2 autoantibodies were found to disappear during periods of clinical remission, but reappeared with disease relapse. No association between anti–NXP‐2 antibody levels and the severity of calcinosis was observed.ConclusionThese findings indicate that anti–NXP‐2 autoantibodies serve as a useful marker for disease activity in patients with IIMs, especially in the absence of calcinosis. The differential associations observed between anti–NXP‐2 autoantibody levels and disease activity suggest that there may be a phenotypic difference between patients with and those without calcinosis.