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Neurology:多发性硬化症免疫病理亚型的临床相关性

2021-09-30 Naomii MedSci原创

研究人员比较多发性硬化患者免疫病理亚型的临床特点,对547例经活检和/或尸检证实的中枢神经系统脱髓鞘患者进行免疫病理分型,进行性疾病与最初的免疫模式关系较小,并提示最终走向与慢性脱髓鞘相关的相似结局

     多发性硬化症(MS)在临床、放射影像及病理上表现出明显的异质性,其病理特征包括多灶性脱髓鞘、炎症、胶质增生和轴突损伤。根据巨噬细胞内是否存在不同的髓鞘降解产物,可将脱髓鞘活性分为早期活跃、晚期活跃、非活跃、早期髓鞘再生和晚期髓鞘再生 (阴影)斑块。急性多发性硬化症斑块的四种不同的病理形式已经被描述。这些免疫表型(IP)在脱髓鞘的效应机制上有所不同:IP-I主要与T细胞/巨噬细胞浸润有关;IP-II补体沉积起重要作用;IP-III代表远端少突胶质细胞病变,丢失髓鞘相关糖蛋白(MAG)和少突胶质细胞凋亡;IP-IV代表原发性少突胶质细胞退行性变。早期活跃的MS斑块的免疫表型因患者而异。临床表现和病理免疫模式之间的关系尚不清楚。由于每个患者在整个病程的所有活跃脱髓鞘病变中都有一种特定的免疫模式,推测这些免疫模式可能与多发性硬化症的不同临床表现或远期预后有关。

      近日,有研究人员对MS患者免疫病理亚型的临床特点进行了比较。

  • 研究人员对547例经活检/尸检证实的MS患者进行免疫病理分型,亚型分布依次为I型(23%)、II型(56%)和III型(22%)。
  • 各免疫表型在尸检/活检年龄(中位年龄41岁,范围4-83岁,p=0.16)和女性占比(54%,p=0.71)方面相似。症状出现后的中位随访时间为2.3年(范围0-38年)。
  • 除了在尸检病例中比例过高(45%比19%在活检队列中,p<0.001),与发作相关的残疾指数在III型比II型更高(EDSS4vs.3的中位数,p=0.02)。
  • 单相程在III型患者中比I型、II型患者更常见(59%:33%:32%,p<0.001)。
  • III型病理的患者与I型或II型的患者相比,当随访≥5年时(总体24%,p=0.49),可能有进展性疾病,尽管有更猛烈的发作表现,但在长期存活率方面没有差异。

      由于活动性病变的数量较少,它们在疾病后期被发现的频率较低,但以上3种免疫亚型都可以在活动性病变中检测到。III型比I型、II型的初始发作更猛烈。无论他们的免疫模式如何,活检的患者似乎都有相似的长期预后。进行性疾病与最初的免疫模式关系较小,并提示最终走向与慢性脱髓鞘相关的相似结局。

文献来源:https://n.neurology.org/content/early/2021/09/09/WNL.0000000000012782.long

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    2022-08-16 jml2009
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    2022-08-24 yinhl1978
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