Monitoring disease activity in juvenile dermatomyositis: the role of von Willebrand factor and muscle enzymes.
Monitoring disease activity in juvenile dermatomyositis: the role of von Willebrand factor and muscle enzymes.
复制标题
监测青少年皮肌炎的疾病活动：冯维勒布兰德因子和肌酶的作用。
作者：
J. Guzman;R. Petty;P. Malleson
目的
评价血浆血管性血友病因子（vWF）和血清肌酶水平的连续测定在检测和预测幼年型皮肌炎（JDM）患儿疾病活动性发作中的作用。
方法
对16例JDM患者进行vWF和肌酶系列测量的回顾性研究，随访540患者月。由对实验室结果设盲的研究者审查图表，疾病发作定义为功能恶化、虚弱加重、肌酶升高、药物需求增加2级。
结果
除2例患者外，所有患者vWF均至少有一次升高，水平高达4.9 IU/ml（正常0.5至1.5）。在23/29例疾病发作中提供了实验室评价。vWF检测疾病发作的敏感性（0.85）和特异性（0.45）并不比LDH或AST更好，即使肌肉酶水平从发作定义中排除。CK和ALT与疾病发作无显著相关性。当AST水平升高> 20%时，发作的风险增加3倍，但vWF不能可靠地预测疾病发作。
结论
虽然vWF与疾病发作有关，但它似乎不能提供比酶测量更多的信息。LDH和AST是最有用的检测耀斑，而AST是唯一的测试能够预测它。
OBJECTIVE
To assess the usefulness of serial measurement of plasma von Willebrand factor (vWF) and serum levels of muscle enzymes in the detection and prediction of flares of disease activity in children with juvenile dermatomyositis (JDM).
METHODS
Retrospective study of serial measurements of vWF and muscle enzymes in 16 patients with JDM followed for 540 patient-months. Charts were reviewed by an investigator blinded to laboratory results, and disease flare was defined as 2 of worsening function, increasing weakness, increasing muscle enzymes, increasing medication requirements.
RESULTS
vWF was increased on at least one occasion in all but 2 patients with levels up to 4.9 IU/ml (normal 0.5 to 1.5). Laboratory evaluations were available in 23/29 disease flares. The sensitivity (0.85) and specificity (0.45) of vWF to detect disease flare were not better than that of LDH or AST even when muscle enzyme levels were excluded from flare definition. CK and ALT were not significantly associated with disease flare. The risk of flare increased 3-fold after a > 20% increase in AST levels, but vWF did not reliably predict disease flare.
CONCLUSION
Although vWF is associated with disease flares, it does not seem to offer more information than enzyme measurements. LDH and AST were most useful in the detection of a flare, while AST was the only test capable of predicting it.