Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
J Neuroimmunol. 2021 Apr 15;353:577518. doi: 10.1016/j.jneuroim.2021.577518. Epub 2021 Feb 10.
Systemic lupus erythematosus (SLE) is a prototype autoimmune disease characterized by circulating autoantibodies and immune complexes involving virtually every organ of the body. However, with respect to central nervous system (CNS), the mechanism of injury is still debated as complement mediated or thrombo-ischemic in nature. We studied the spectrum of neuropathological changes in twelve autopsy cases of SLE and evaluated the role of immune-complexes and complement activation in contributing to the thrombo-ischemic injury and correlated these features with clinical profile.
Autopsy records of all cases of SLE over a period of 20 years (2000-2019) were reviewed. Clinical history including neuropsychiatric symptoms and detailed histopathological analysis was performed. Direct immunofluorescence for IgM, IgG, IgA, C1q, C3, C4d, Kappa, Lambda and immunohistochemistry for C5b-9 was performed on lesional areas in paraffin embedded brain sections. Control tissue from brain was taken from two patients who died of sudden cardiac event.
Our cohort comprised of 12 cases with age range from 12 to 40 years and all were female patients. Microinfarction and vasculopathy seen in eight cases were the commonest findings. Four cases with microinfarcts had non-bacterial thrombotic endocarditis in heart. Microthrombi adjacent to microinfarcts were seen in 4 cases. Variable deposition of immunoglobulins (predominantly IgG) and complements (C1q, C3, C4d) was evident in cortical arterioles (2 cases) and small capillaries (1 case). Neurological symptoms were seen in four cases, of which, three had associated invasive fungal infection with secondary vasculitis. Active lupus vasculitis was identified in a single case. C5b-9 immunoexpression was not detected in any of the cases.
Our study adds observational data to the existing literature that the predominant neuropathological features of SLE are related to thrombo-ischemic injury and small vasculopathic changes. Only in a minor subset (25%), it is mediated by immune-complexes and complements. Immune-complex deposition on immunofluorescence in cortical vessels (cerebral lupus vasculopathy) is a novel finding which has not been reported earlier.
系统性红斑狼疮（SLE）是一种典型的自身免疫性疾病，其特征为循环自身抗体和免疫复合物涉及身体的几乎所有器官。然而，就中枢神经系统（CNS）而言，其损伤机制仍存在争议，是补体介导还是血栓-缺血性。我们研究了 12 例尸检 SLE 病例的神经病理学变化谱，并评估了免疫复合物和补体激活在导致血栓-缺血性损伤中的作用，并将这些特征与临床特征相关联。
回顾了过去 20 年（2000-2019 年）期间所有 SLE 病例的尸检记录。进行了包括神经精神症状在内的临床病史和详细的组织病理学分析。对石蜡包埋脑切片中的病变区域进行直接免疫荧光法检测 IgM、IgG、IgA、C1q、C3、C4d、Kappa、Lambda 和 C5b-9 的免疫组织化学染色。从两名死于心脏骤停的患者的脑控制组织中获取组织。
我们的队列包括 12 例年龄在 12 至 40 岁之间的女性患者。在 8 例中最常见的发现是微梗塞和血管病变。在 4 例有微梗塞的病例中，心脏有非细菌性血栓性心内膜炎。在 4 例微梗塞附近可见微血栓。在皮质小动脉（2 例）和小毛细血管（1 例）中可见免疫球蛋白（主要是 IgG）和补体（C1q、C3、C4d）的可变沉积。在 4 例中有神经症状，其中 3 例伴有侵袭性真菌感染和继发性血管炎。在 1 例中有活动性狼疮血管炎。在任何病例中均未检测到 C5b-9 免疫表达。
我们的研究增加了现有文献的观察数据，即 SLE 的主要神经病理学特征与血栓-缺血性损伤和小血管病变有关。只有在一小部分（25%）病例中，是由免疫复合物和补体介导的。免疫荧光在皮质血管中的免疫复合物沉积（脑狼疮血管病）是一个新的发现，以前没有报道过。
