THE RELATIONSHIP OF COMPLEMENT-MEDIATED MICROVASCULOPATHY TO THE HISTOLOGIC FEATURES AND CLINICAL DURATION OF DISEASE IN DERMATOMYOSITIS
THE RELATIONSHIP OF COMPLEMENT-MEDIATED MICROVASCULOPATHY TO THE HISTOLOGIC FEATURES AND CLINICAL DURATION OF DISEASE IN DERMATOMYOSITIS
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DOI：
10.1001/archneur.1991.00530130034016
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发表时间：
1991-01-01
影响因子：
--
通讯作者：
MENDELL, JR
中科院分区：
文献类型：
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作者：
KISSEL, JT;HALTERMAN, RK;MENDELL, JR
越来越多的证据表明补体介导的微血管病变可能在皮肌炎中起致病作用。 在之前的一项研究中，我们证明了儿童和成人皮肌炎患者肌肉微血管中存在C5 b-9补体膜攻击复合物的新抗原。 为了进一步描述血管补体沉积和组织学变化之间的关系，对39例皮肌炎活检标本（26例成人，13例儿童）进行了定量组织病理学分析。 具有局灶性肌原纤维损失的纤维束的百分比（在缺血性肌纤维损伤的演变早期观察到的变化）与具有膜攻击复合物的毛细血管沉积物的纤维束的百分比之间存在显著相关性。 相反，在具有较高百分比的束周围萎缩的束的活检标本中，膜攻击复合物沉积物明显不太常见。 逐分册分析支持这些意见。 活检标本微血管膜攻击复合体阴性的患者比血管补体沉积的患者临床虚弱的时间明显更长。 这些数据支持补体介导的血管病变是皮肌炎肌肉病变演变中的主要免疫病理事件的假设。
Accumulating evidence indicates that a complement-mediated microvasculopathy may play a pathogenic role in dermatomyositis. In a previous study, we demonstrated neoantigens of the C5b-9 complement membrane attack complex in the muscle microvasculature of childhood and adult cases of dermatomyositis. To further characterize the relationship between the vascular complement deposits and histologic changes, quantitative histopathologic analyses were performed on 39 dermatomyositis biopsy specimens (26 adult, 13 children). There was a significant correlation between the percentage of fascicles with fibers having focal myofibrillar loss, a change seen early in the evolution of ischemic muscle fiber damage, and the percentage of fascicles having capillary deposits of membrane attack complex. Conversely, in biopsy specimens with a higher percentage of fascicles with peri-fascicular atrophy, membrane attack complex deposits were significantly less common. A fascicle-by-fascicle analysis supported these observations. Patients whose biopsy specimens were negative for microvascular membrane attack complex had clinical weakness for a significantly longer time than those patients with vascular complement deposits. These data support the hypothesis that the complement-mediated vasculopathy is a primary immunopathogenic event in the evolution of muscle lesions in dermatomyositis.