Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP)
Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP)
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DOI：
10.1007/s004010050941
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发表时间：
1998-12-01
影响因子：
12.7
通讯作者：
Rizzuto, SG
中科院分区：
文献类型：
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作者：
Rizzuto, N;Morbin, M;Rizzuto, SG
在一项旨在确定慢性炎症性脱髓鞘性多发性神经病 (CIDP) 典型神经病理学特征的研究中，我们回顾了 105 名患有这种疾病的患者的腓肠神经活检结果。活检时患者的平均年龄为 49 岁。 65% 的患者的疾病呈进展性，35% 的患者呈复发缓解型。 47% 的病例是特发性的；其余的人有各种并发条件。所有腓肠神经活检标本均显示不同程度的与洋葱球相关的活动性脱髓鞘（48％的病例）、神经内膜水肿（55％）和炎症浸润（25％）。免疫病理学标志是 T 细胞浸润，伴有巨噬细胞激活和主要组织相容性复合体 (MHC) II 类表达上调，但髓鞘上没有 B 细胞浸润或免疫球蛋白沉积。在 30% 的病例中，一些髓鞘显示出 C3d 沉积。促炎细胞因子表达分析始终显示，血管周围和神经内膜分支细胞中存在白细胞介素-1，神经外膜巨噬细胞中普遍存在肿瘤坏死因子-α，而仅在血管周围炎症细胞的样本中检测到干扰素-γ。这种免疫学模式表明免疫的细胞成分在 CIDP 中起主要作用。在 19% 的病例中，神经病理学变化呈局灶性分布。这一独特的特征与更活跃的脱髓鞘、更频繁地检测到炎症浸润和更显着的免疫激活相对应，表明局灶性受累是疾病过程中可能的一步。
In a study designed to identify the neuropathological features typical of chronic inflammatory demyelinating polyneuropathy (CIDP), we reviewed the sural nerve biopsy findings in 105 patients with this disorder. The patients' mean age at biopsy was 49 years. In 65% of patients the disease had a progressive and in 35% a relapsing-remitting course. In 47% of cases the disorder was idiopathic; the remainder had various concurrent conditions. All sural nerve biopsy specimens showed varying amounts of active demyelination associated with onion bulbs (48% of cases), endoneurial edema (55%) and inflammatory infiltrates (25%). The immunopathological hallmarks were T cell infiltration with macrophagic activation and up-regulation of major histocompatibility complex (MHC) class II expression, without B cell infiltration or immunoglobulin deposition on myelin sheaths. In 30% of cases some myelin sheaths showed C3d deposition. Analysis of proinflammatory cytokine expression invariably showed interleukin-1 in perivascular and endoneurial ramified cells and tumor necrosis factor-alpha prevalently in epineurial macrophages, whereas it detected interferon-gamma only in samples with perivascular inflammatory cells. This immunological pattern suggests that the cellular components of immunity play the major role in CIDP. In 19% of cases the neuropathological changes had a focal distribution. This distinctive feature corresponded to more active demyelination, more frequent detection of inflammatory infiltrates and more prominent immunological activation, suggesting that focal involvement is a possible step in the course of the disease.