Disseminated perivenous necrotizing encephalomyelitis in systemic lupus erythematosus: report of an autopsy case
Disseminated perivenous necrotizing encephalomyelitis in systemic lupus erythematosus: report of an autopsy case
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系统性红斑狼疮并发播散性静脉周围坏死性脑脊髓炎一例尸检报告
作者：
M. Shintaku;R. Matsumoto
患者为22岁女性，因系统性红斑狼疮(SLE)治疗10年，因关节痛、黑便、行走困难入院治疗。头颅CT检查可见散在分布于脑白质和基底节的颗粒状高密度病变。尸检发现，静脉周围可见多个边界清楚的棕色变色灶，散在分布于大脑白质和基底节。病理组织学上，这些病变由围绕静脉的凝固性坏死灶组成。病灶内的静脉呈纤维性增厚，但无脉管炎的迹象。在病变的边缘，轴突比它们的髓鞘保存得更好。本病例的神经病理表现与急性播散性(静脉周围)脑脊髓炎非常相似，尽管炎性细胞浸润明显已经消退。尽管其发病机制尚不清楚，但这一发现不应被视为偶然的并发症，而应被视为SLE中发生的一种罕见的中枢神经系统损害亚型。
Abstract The patient, a 22-year-old woman who had been treated for systemic lupus erythematosus (SLE) for 10 years, was hospitalized for arthralgia, melena, and difficulty in walking. CT examination of the brain showed grain-like high-density lesions scattered throughout the cerebral white matter and basal ganglia. At autopsy, multiple perivenous, well-demarcated foci of brownish discoloration were seen scattered throughout the cerebral white matter and basal ganglia. Histopathologically these lesions consisted of foci of coagulation necrosis surrounding the veins. The veins in the foci showed fibrous thickening of the walls, but there were no indications of vasculitis. At the periphery of the lesions, the axons were better preserved than their myelin sheaths. The neuropathological findings in the present case closely resemble those of acute disseminated (perivenous) encephalomyelitis, although an inflammatory cell infiltration had apparently already subsided. Although its pathogenesis remains unclear, this finding should not be regarded as an incidental complication but rather as a rare subtype of central nervous system lesion occurring with SLE.
系统性红斑狼疮中枢神经系统表现的发病机制。
影响因子：
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作者：
Zvaifler,NJ;Bluestein,HG
通讯作者：
Bluestein,HG