Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease.
Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease.
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DOI：
10.1136/annrheumdis-2013-203396
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发表时间：
2015-01
影响因子：
27.4
通讯作者：
UK Juvenile Dermatomyositis Research Group
中科院分区：
文献类型：
--
作者：
Varsani H;Charman SC;Li CK;Marie SK;Amato AA;Banwell B;Bove KE;Corse AM;Emslie-Smith AM;Jacques TS;Lundberg IE;Minetti C;Nennesmo I;Rushing EJ;Sallum AM;Sewry C;Pilkington CA;Holton JL;Wedderburn LR;UK Juvenile Dermatomyositis Research Group
研究青少年皮肌炎（JDM）患者的肌肉活检组织，以测试一种评分工具的可靠性，该工具设计用于量化除股四头肌外肱骨二头肌组织学异常的严重程度。此外，评估该工具的要素是否与疾病严重程度的临床测量相关。纳入55例肌肉活检组织和临床资料的JDM患者。活检样本（33个股四头肌，22个二头肌）制备并使用标准化方案进行染色。国际青少年皮肌炎活检共识组使用拉丁方设计，使用我们之前发表的评分工具对病例进行评分。信度采用类内相关系数（ICC）和评分者一致性（α）来评估。来自最可靠工具项目的评分与活检时疾病活动性的临床测量相关。对于许多工具项目，包括使用视觉模拟量表对严重程度的总体评估，观察者之间和内部的一致性是好的或高的。该工具在二头肌和股四头肌样本上同样有效。使用最可靠评分项目的改进工具显示与疾病活动的测量具有良好的相关性。JDM活检评分工具具有高度的观察者间和内部一致性，可用于肱二头肌和股四头肌肌肉组织。重要的是，修改后的工具与疾病活动性的临床测量有很好的相关性。我们建议在JDM的诊断调查和临床试验中应考虑对肌肉活检组织进行标准化评估。
To study muscle biopsy tissue from patients with juvenile dermatomyositis (JDM) in order to test the reliability of a score tool designed to quantify the severity of histological abnormalities when applied to biceps humeri in addition to quadriceps femoris. Additionally, to evaluate whether elements of the tool correlate with clinical measures of disease severity. 55 patients with JDM with muscle biopsy tissue and clinical data available were included. Biopsy samples (33 quadriceps, 22 biceps) were prepared and stained using standardised protocols. A Latin square design was used by the International Juvenile Dermatomyositis Biopsy Consensus Group to score cases using our previously published score tool. Reliability was assessed by intraclass correlation coefficient (ICC) and scorer agreement (α) by assessing variation in scorers’ ratings. Scores from the most reliable tool items correlated with clinical measures of disease activity at the time of biopsy. Inter- and intraobserver agreement was good or high for many tool items, including overall assessment of severity using a Visual Analogue Scale. The tool functioned equally well on biceps and quadriceps samples. A modified tool using the most reliable score items showed good correlation with measures of disease activity. The JDM biopsy score tool has high inter- and intraobserver agreement and can be used on both biceps and quadriceps muscle tissue. Importantly, the modified tool correlates well with clinical measures of disease activity. We propose that standardised assessment of muscle biopsy tissue should be considered in diagnostic investigation and clinical trials in JDM.
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