伴髓鞘少突胶质细胞糖蛋白自身抗体的中枢神经系统炎性脱髓鞘病病理.,Acta Neuropathologica

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伴髓鞘少突胶质细胞糖蛋白自身抗体的中枢神经系统炎性脱髓鞘病病理.
Acta Neuropathologica ( IF 9.3 ) Pub Date : 2020-02-11 , DOI: 10.1007/s00401-020-02132-y
Romana Höftberger ₁ , Yong Guo ₂ , Eoin P Flanagan _{2,

3} , A Sebastian Lopez-Chiriboga ₂ , Verena Endmayr ₁ , Sonja Hochmeister ₄ , Damir Joldic ₅ , Sean J Pittock _{2,

3} , Jan Mendelt Tillema ₂ , Mark Gorman ₆ , Hans Lassmann ₇ , Claudia F Lucchinetti ₂

Affiliation

我们试图在档案尸检/活检队列中定义髓鞘少突胶质细胞糖蛋白 (MOG) 抗体相关疾病 (MOGAD) 的病理特征。我们通过基于活细胞的检测方法对具有构象形式的全长 MOG 的 CNS 炎性脱髓鞘疾病患者的 MOG 抗体血清阳性的 2 份尸检和 22 份脑活检进行了组织病理学分析。MOGAD 尸检（52 岁和 67 岁）展示了在 22 次脑活检（中位年龄，10 岁；范围，1-66；56% 女性）中观察到的所有组织病理学特征。临床、放射学和实验室特征和病程（78% 复发）与 MOGAD 一致。MOGAD 病理以静脉周围和融合的白质脱髓鞘共存为主，与典型的 MS 相比，皮质内脱髓鞘病变过多。不存在如 MS 中所见的放射状扩张汇合缓慢扩张的白质中的阴燃病变。CD4+ T 细胞占主导地位的炎症反应与粒细胞浸润占主导地位。补体沉积存在于所有活动性白质病变中，但未观察到 MOG 的优先丢失。AQP4 被保留，没有营养不良的星形胶质细胞，以及可变的少突胶质细胞和轴突破坏。MOGAD 在病理上与 AQP4-IgG 血清阳性 NMOSD 不同，但与 MS 和 ADEM 有一些重叠的特征，表明是一种过渡性病理。在没有选择性 MOG 蛋白丢失的情况下补体沉积表明涉及体液机制，然而，反对 MOG 抗原的内吞作用。与 MOG-EAE 的相似之处表明 MOG 可能是增强 CNS 脱髓鞘的放大因子，可能是通过补体介导的髓鞘破坏或 ADCC 吞噬作用。

"点击查看英文标题和摘要"

The pathology of central nervous system inflammatory demyelinating disease accompanying myelin oligodendrocyte glycoprotein autoantibody.

We sought to define the pathological features of myelin oligodendrocyte glycoprotein (MOG) antibody associated disorders (MOGAD) in an archival autopsy/biopsy cohort. We histopathologically analyzed 2 autopsies and 22 brain biopsies from patients with CNS inflammatory demyelinating diseases seropositive for MOG-antibody by live-cell-based-assay with full length MOG in its conformational form. MOGAD autopsies (ages 52 and 67) demonstrate the full spectrum of histopathological features observed within the 22 brain biopsies (median age, 10 years; range, 1-66; 56% female). Clinical, radiologic, and laboratory characteristics and course (78% relapsing) are consistent with MOGAD. MOGAD pathology is dominated by coexistence of both perivenous and confluent white matter demyelination, with an over-representation of intracortical demyelinated lesions compared to typical MS. Radially expanding confluent slowly expanding smoldering lesions in the white matter as seen in MS, are not present. A CD4+ T-cell dominated inflammatory reaction with granulocytic infiltration predominates. Complement deposition is present in all active white matter lesions, but a preferential loss of MOG is not observed. AQP4 is preserved, with absence of dystrophic astrocytes, and variable oligodendrocyte and axonal destruction. MOGAD is pathologically distinguished from AQP4-IgG seropositive NMOSD, but shares some overlapping features with both MS and ADEM, suggesting a transitional pathology. Complement deposition in the absence of selective MOG protein loss suggest humoral mechanisms are involved, however argue against endocytic internalization of the MOG antigen. Parallels with MOG-EAE suggest MOG may be an amplification factor that augments CNS demyelination, possibly via complement mediated destruction of myelin or ADCC phagocytosis.

更新日期：2020-04-23

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