Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Acta Neurol Scand. 2011 Feb;123(2):122-9. doi: 10.1111/j.1600-0404.2010.01353.x.
Currently there is no reliable diagnostic marker to distinguish between the subgroups of idiopathic inflammatory myopathies (IIMs), i.e. dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM). Membrane attack complex (MAC) has been shown to be involved in the pathogenesis of dermatomyositis but its role as a diagnostic marker has not been evaluated.
To assess the diagnostic utility of MAC deposition in distinguishing dermatomyositis from other neuromuscular disorders.
Immunohistochemical detection of MAC deposition on endomysial microvessels was carried out on 127 muscle biopsies comprising of 21 cases of dermatomyositis, 42 other IIMs and 64 non-IIM neuromuscular diseases.
MAC deposition showed a high sensitivity (80.9%) and specificity (85%) to differentiate DM from other IIMs. Its specificity was higher (98.4%) in discriminating DM from non-IIM muscular diseases and IIM from non-IIMs.
MAC deposition can serve as a reliable marker to distinguish DM from other IIMs (i.e. PM and IBM) as well as from non-IIM diseases. It can also serve as a useful adjunct in diagnosis of IIMs when there is diagnostic dilemma with their morphologic similarities. These results provide further credence to the long-standing view that MAC-mediated capillary destruction is involved in the immunopathogenesis of DM.
目前尚无可靠的诊断标志物来区分特发性炎性肌病(IIM)的亚组,即皮肌炎(DM)、多发性肌炎(PM)和包涵体肌炎(IBM)。已表明膜攻击复合物(MAC)参与了皮肌炎的发病机制,但尚未评估其作为诊断标志物的作用。
评估 MAC 沉积在区分皮肌炎与其他神经肌肉疾病方面的诊断效用。
对 127 例肌肉活检进行了 MAC 沉积在内肌膜微血管上的免疫组织化学检测,包括 21 例皮肌炎、42 例其他 IIM 和 64 例非 IIM 神经肌肉疾病。
MAC 沉积对区分 DM 与其他 IIM 具有高敏感性(80.9%)和特异性(85%)。其特异性(98.4%)在区分 DM 与非 IIM 肌肉疾病以及 IIM 与非 IIMs 方面更高。
MAC 沉积可作为一种可靠的标志物,用于区分 DM 与其他 IIM(即 PM 和 IBM)以及非 IIM 疾病。当 IIM 的形态学相似导致诊断困境时,它也可以作为诊断 IIM 的有用辅助手段。这些结果进一步证实了 MAC 介导的毛细血管破坏参与皮肌炎发病机制的长期观点。