Wedderburn Lucy R, Varsani Hemlata, Li Charles K C, Newton Katy R, Amato Anthony A, Banwell Brenda, Bove Kevin E, Corse Andrea M, Emslie-Smith Alison, Harding Brian, Hoogendijk Jessica, Lundberg Ingrid E, Marie Suely, Minetti Carlo, Nennesmo Inger, Rushing Elisabeth J, Sewry Caroline, Charman Susan C, Pilkington Clarissa A, Holton Janice L
University College London, London, UK.
Arthritis Rheum. 2007 Oct 15;57(7):1192-201. doi: 10.1002/art.23012.
To devise and test a system with which to evaluate abnormalities on muscle biopsy samples obtained from children diagnosed with juvenile dermatomyositis (DM).
We established an International Consensus Group on Juvenile DM Biopsy and carried out 2 phases of consensus process and scoring workshops. Biopsy sections (n = 33) were stained by standard methods. The scoring tool was based on 4 domains of change: inflammatory, vascular, muscle fiber, and connective tissue. Using a Latin square design, biopsy samples were scored by 11 experts for items in each domain, and for a global abnormality measure using a 10-cm visual analog score (VAS 0-10). The tool's reliability was assessed using an intraclass correlation coefficient (ICC) and scorer agreement (alpha) by determining variation in scorers' ratings.
There was good agreement in many items of the tool, and several items refined between the meetings improved in reliability and/or agreement. The inflammatory and muscle fiber domains had the highest reliability and agreement. The overall VAS score for abnormality had high agreement and reliability, reaching an ICC of 0.863 at the second consensus meeting.
We propose a provisional scoring system to measure abnormalities on muscle biopsy samples obtained from children with juvenile DM. This system needs to be validated, and then could be used in prospective studies to test which features of muscle pathology are prognostic of disease course or outcome. We suggest that the process we used could be a template for developing similar systems in other forms of myositis.
设计并测试一种系统，用于评估从被诊断为幼年皮肌炎（DM）的儿童身上获取的肌肉活检样本中的异常情况。
我们成立了一个关于幼年DM活检的国际共识小组，并开展了两个阶段的共识过程和评分研讨会。活检切片（n = 33）采用标准方法进行染色。评分工具基于四个变化领域：炎症、血管、肌纤维和结缔组织。采用拉丁方设计，11位专家对活检样本在每个领域的项目进行评分，并使用10厘米视觉模拟评分法（VAS 0 - 10）对整体异常程度进行评分。通过确定评分者评分的差异，使用组内相关系数（ICC）和评分者一致性（α）来评估该工具的可靠性。
该工具的许多项目具有良好的一致性，会议期间完善的几个项目在可靠性和/或一致性方面有所提高。炎症和肌纤维领域具有最高的可靠性和一致性。异常情况的总体VAS评分具有较高的一致性和可靠性，在第二次共识会议上达到了0.863的ICC。
我们提出了一种临时评分系统，用于测量从患有幼年DM的儿童身上获取的肌肉活检样本中的异常情况。该系统需要进行验证，然后可用于前瞻性研究，以测试肌肉病理学的哪些特征对疾病进程或结局具有预后意义。我们建议我们所使用的过程可以作为开发其他形式肌炎类似系统的模板。
