Zhang Nan, Cao Jie, Zhao Meng, Sun Li
Department of Neurology and Neuroscience Center, the First Hospital of Jilin University, Changchun, Jilin Department of Neurology, Yidu Central Hospital, Weifang, Shandong, China.
Medicine (Baltimore). 2017 Dec;96(49):e9037. doi: 10.1097/MD.0000000000009037.
Systemic lupus erythematosus (SLE) is an autoimmune inflammatory connective tissue disorder. It may cause neurologic damage which is mainly characterized by central and mental system, while peripheral sexual damage is relatively rare in which Guillain-Barré Syndrome (GBS) as the first performance is more rare . GBS is an autoimmune peripheral neuropathy usually triggered by an antecedent bacterial or viral infection, with SLE being a rare cause.
A 65-year-old male presented to the hospital with progressive numbness and adynamia in extremities. His vital signs were stable. 5 days later, his condition aggravated and mechanical ventilation was necessitated owing to severe dyspnea.
Based on the clinical symptoms and results of the lumbar puncture and electromyography, he was first diagnosed as GBS, however, after treatment his condition was deteriorate and the blood test showed abnormal immune indices, then renal biopsy was performed, which confirmed the diagnosis of peripheral nervous system in patients with systemic lupus erythematosus (PNS-SLE).
Firstly he was treated with intravenous immunoglobulin (IVIG) for 5 days. After his condition deterioration, he was conducted endotracheal intubation and, finally, a tracheostomy was performed. Later on he was treated with steroid therapy for several weeks.
The patient showed remarkable recovery and was able to walk on his own by the time of discharge.
PNS-SLE can, by itself, be one of the main causes of morbidity and mortality. Electromyography and renal biopsy should be considered when relevant. Peripheral neuropathy in SLE should be given greater recognition, and rarer forms of presentation should be taken seriously in the differential diagnosis when the clinical picture is atypical. Glucocorticoids may play an important role in the treatment of PNS-SLE.
系统性红斑狼疮（SLE）是一种自身免疫性炎症性结缔组织疾病。它可能导致神经损伤，主要表现为中枢和精神系统病变，而周围神经损伤相对少见，以格林-巴利综合征（GBS）为首发表现的更为罕见。GBS是一种自身免疫性周围神经病，通常由先前的细菌或病毒感染引发，SLE是其罕见病因。
一名65岁男性因四肢进行性麻木和无力入院。生命体征平稳。5天后病情加重，因严重呼吸困难需要机械通气。
根据临床症状及腰椎穿刺和肌电图检查结果，最初诊断为GBS，但治疗后病情恶化，血液检查显示免疫指标异常，随后进行肾活检，确诊为系统性红斑狼疮伴发周围神经系统病变（PNS-SLE）。
首先给予静脉注射免疫球蛋白（IVIG）治疗5天。病情恶化后进行气管插管，最终行气管切开术。之后接受了数周的类固醇治疗。
患者恢复显著，出院时能够自行行走。
PNS-SLE本身可能是发病和死亡的主要原因之一。相关时应考虑进行肌电图检查和肾活检。SLE中的周围神经病变应得到更多认识，临床症状不典型时，在鉴别诊断中应重视罕见的表现形式。糖皮质激素可能在PNS-SLE的治疗中发挥重要作用。
