系统性红斑狼疮的慢性炎症性脱髓鞘性多神经病变:一种罕见的实体

R. Mohd, F. Nordin, Rizna Cader
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引用次数: 1

摘要

系统性红斑狼疮(SLE)的神经系统表现各不相同,通常影响中枢神经系统(CNS)而不是周围神经系统。神经精神或中枢神经系统的表现可高达24-54%,而周围神经系统的表现则低至5-27%左右。慢性炎症性脱髓鞘性多神经根病(CIDP)是SLE患者三种最常见的外周神经系统病变之一,并导致严重的衰弱效应。然而,鲜有报道。我们对2000年至2015年间诊断为CIDP的所有SLE患者进行回顾性研究,并对2000年至2015年间诊断为CIDP的患者进行随访。我们回顾了他们的医疗记录,分析了他们的临床表现、调查、治疗、治疗反应和任何神经系统后遗症。共审查了512份病例记录。这4例患者中有3例女性,1例男性,年龄在26至46岁之间。3例表现为横贯脊髓炎,1例表现为急性运动和感觉轴索神经病变。所有患者均接受大剂量皮质类固醇治疗,3例患者接受环磷酰胺治疗,另1例患者接受霉酚酸酯诱导。1例患者完全恢复,2例有持续但改善的麻木,另1例有残余无力。SLE累及周围神经系统可导致严重的衰弱效应。早期诊断和治疗是限制神经系统后遗症的关键。
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Chronic Inflammatory Demyelinating Polyneuropathy in Systemic Lupus Erythematosus: A Rare Entity
Neurological manifestations in Systemic Lupus Erythematous (SLE) varies and commonly affects the Central Nervous System (CNS) rather than the peripheral nervous system. Neuropsychiatric or CNS manifestation can be as high as 24-54%, whereas the peripheral nervous system involvement is lower around 5-27%. Chronic Inflammatory Demyelinating Polyradiculopathy (CIDP) is one of the three commonest peripheral nervous system involvements in SLE patients and results with severe debilitating effects. However, it is rarely reported.A retrospective review of all SLE patients that were diagnosed with CIDP between 2000 and 2015 was done under follow up at our center that were diagnosed with CIDP between 2000 and 2015. We reviewed their medical records and analyzed their clinical presentation, investigations, treatment instituted, response to therapy and any neurological sequealae.A total of 512 case notes were reviewed. Of these 4 patients presented with CIDP (3 females, 1 male) aged between 26 to 46 years old. Three presented with transverse myelitis and the other one with acute motor and sensory axonal neuropathy. All patients were treated with high dose corticosteroids, three patients received cyclophosphamide whilst the other patient was induced with mycophenolate mofetil. Complete recovery was seen in one patient, two had persistent but improving numbness and the other one had a residual weakness.Peripheral nervous system involvement in SLE can result in serious debilitating effects. Early diagnosis and treatment are crucial in limiting the neurological sequealae.
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