Kiefer R, Kieseier B C, Brück W, Hartung H P, Toyka K V
Department of Neurology, University of Würzburg, Germany.
Brain. 1998 Mar;121 ( Pt 3):469-79. doi: 10.1093/brain/121.3.469.
The pathological differential diagnosis between potentially treatable autoimmune neuropathies and degenerative neuropathies is often difficult if major T-cell infiltrates are absent in sural nerve biopsies. Since it is suggested that macrophages play a central pathogenetic role in inflammatory neuropathies, we investigated the expression of macrophage differentiation antigens associated with acute (MRP14 and 27E10 antigens) and more chronic inflammation (MRP8 and 25F9 antigens) in 76 sural nerve biopsies from patients with Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, other inflammatory neuropathies, hereditary neuropathies and normal sural nerves. Macrophage differentiation antigens were immunocytochemically detected in a majority of inflammatory biopsies but rarely in non-inflammatory disease controls and normal-looking nerves. Quantification of labelled endoneurial cells revealed significantly elevated cell counts compared with controls. In individual biopsies, elevated levels of one differentiation antigen were not necessarily associated with high expression of the other antigens, pointing to functional heterogeneity of endoneurial macrophages. Endoneurial cell counts for at least one of the differentiation markers that were greater than in any of the non-inflammatory control nerves were found in two-thirds of all inflammatory biopsies, whereas T-cell counts and in particular total macrophage counts were less sensitive in picking up biopsies from patients with inflammatory neuropathies. Antibodies to macrophage differentiation antigens are additional simple and helpful diagnostic tools in differentiating autoimmune from non-inflammatory neuropathies in sural nerve biopsies.
如果腓肠神经活检中没有主要的T细胞浸润，那么在潜在可治疗的自身免疫性神经病和退行性神经病之间进行病理鉴别诊断通常很困难。由于有研究表明巨噬细胞在炎性神经病中起核心致病作用，我们调查了76例来自吉兰 - 巴雷综合征、慢性炎性脱髓鞘性多发性神经病、其他炎性神经病、遗传性神经病患者以及正常腓肠神经的活检样本中，与急性炎症（MRP14和27E10抗原）和更慢性炎症（MRP8和25F9抗原）相关的巨噬细胞分化抗原的表达情况。巨噬细胞分化抗原在大多数炎性活检样本中通过免疫细胞化学检测到，但在非炎性疾病对照和外观正常的神经中很少检测到。对标记的神经内膜细胞进行定量分析发现，与对照组相比，细胞计数显著升高。在单个活检样本中，一种分化抗原水平升高不一定与其他抗原的高表达相关，这表明神经内膜巨噬细胞存在功能异质性。在所有炎性活检样本的三分之二中，发现至少一种分化标志物的神经内膜细胞计数高于任何非炎性对照神经，而T细胞计数，特别是总巨噬细胞计数在识别炎性神经病患者的活检样本时敏感性较低。巨噬细胞分化抗原抗体是在腓肠神经活检中区分自身免疫性神经病和非炎性神经病的额外简单且有用的诊断工具。
