From Ghent University Hospital (P.A.O., B.D.P., R.C., J.L.D.B.), Belgium; Academic Medical Centre (E.A., F.B., M.d.V.), University of Amsterdam, the Netherlands; Harvard Medical School (A.A.), Boston, MA; CHU Henri Mondor (D.D.), Créteil, France; Hospital Santa Creu i Sant Pau (E.G.), Barcelona, Spain; Université Paris Est-Créteil (R.G.), France; Charité-Universitätsmedizin Berlin (H.-H.G., W.S.), Germany; John Radcliffe Hospital (D.H.-J.); Oxford University Hospitals (M.H.); UCL Institute of Neurology (J.H.), London, UK; Syddansk Universitet (H.d.S.), Odense, Denmark; and Mayo Clinic (D.S.), Rochester, MN.
Neurology. 2019 Aug 27;93(9):e889-e894. doi: 10.1212/WNL.0000000000008005. Epub 2019 Jul 29.
To determine interrater variability in diagnosing individual muscle biopsy abnormalities and diagnosis.
We developed a scoring tool to analyze consensus in muscle biopsy reading of an ad hoc workgroup of international experts. Twenty-four samples from patients with suspected idiopathic inflammatory myopathy (IIM) were randomly selected, providing sections that were stained with standard histologic and immunohistochemical methods. Sections were made available on an online platform, and experts were queried about myopathologic features within 4 pathologic domains: muscle fibers, inflammation, connective tissue, and vasculature. A short clinical presentation of cases was included, and experts were asked to give a tentative diagnosis of polymyositis, dermatomyositis, inclusion-body myositis, antisynthetase syndrome-related myositis, immune-mediated necrotizing myopathy, nonspecific myositis, or other disease. Fleiss κ values, scoring interrater variability, showed the highest agreement within the muscle fiber and connective tissue domains.
Despite overall low κ values, moderate agreement was achieved for tentative diagnosis, supporting the idea of using holistic muscle biopsy interpretation rather than adding up individual features.
The assessment of individual pathologic features needs to be standardized and harmonized and should be measured for sensitivity and specificity for subgroup classification. Standardizing the process of diagnostic muscle biopsy reading would allow identification of more homogeneous patient cohorts for upcoming treatment trials.
确定诊断个体肌肉活检异常和诊断的观察者间变异性。
我们开发了一种评分工具，用于分析一组国际专家的肌肉活检阅读共识。随机选择了 24 名疑似特发性炎性肌病（IIM）患者的样本，提供了用标准组织学和免疫组织化学方法染色的切片。将切片放在在线平台上，专家们被询问了 4 个病理域内的肌病特征：肌肉纤维、炎症、结缔组织和脉管系统。还包括了病例的简短临床介绍，并要求专家们对多发性肌炎、皮肌炎、包涵体肌炎、抗合成酶综合征相关肌炎、免疫介导性坏死性肌病、非特异性肌炎或其他疾病做出暂定诊断。Fleiss κ 值，评分观察者间变异性，在肌肉纤维和结缔组织域内显示出最高的一致性。
尽管总体 κ 值较低，但暂定诊断达成了中等程度的一致性，支持使用整体肌肉活检解释而不是累加个别特征的想法。
需要对个体病理特征进行标准化和协调，并应针对亚组分类测量其敏感性和特异性。标准化诊断性肌肉活检阅读过程将有助于为即将到来的治疗试验确定更同质的患者队列。
