Javadi Parvaneh Vadood, Jari Mohsen, Qhasemi Saeideh, Nasehi Mohammad Mahdi, Rahmani Khosro, Shiari Reza
Mofid Children's Hospital, Shahid Beheshti Medical University of Sciences, Tehran, Iran.
Open Access Rheumatol. 2019 Apr 24;11:97-101. doi: 10.2147/OARRR.S204109. eCollection 2019.
Systemic lupus erythematosus (SLE) is an autoimmune disease which involves multiple organs, including peripheral nervous system. We describe a 12-year-old boy with progressively worsening neurological symptoms as first manifestation. Legs pain, loss of balance, and lower extremity weakness were the reason for his admission in neurologic ward. The patient was started on intravenous immunoglobulin therapy due to the possibility of Guillain-Barre syndrome and acute inflammatory demyelinating polyneuropathy (AIDP). However, there was no appropriate response and he developed recurrent attacks of polyneuropathy again with diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Then, he received intravenous pulse of methylprednisolone for 5 consecutive days followed by oral prednisolone for 3 months. One month after withdrawal of corticosteroid he admitted again with the same manifestations. Rheumatologic workup revealed the presence of leukopenia, hemolytic anemia, hematuria, proteinuria, positive antinuclear antibodies, and ds-DNA antibodies. On the basis of the American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria for SLE, the patient had underlying diagnosis of SLE. Eventually, he was treated by the pulse of methylprednisolone and cyclophosphamide, and oral hydroxychloroquine and prednisolone. His neurological and physical symptoms improved and complete neurological recovery occurred several months later. SLE and AIDP/CIDP are different entities, but ADP/CIDP can be part of the neurologic manifestations of the SLE. Although the association between AIDP/CIDP and SLE is very rare especially as a first manifestation of SLE, it should be early recognized for rapid appropriate treatment.
系统性红斑狼疮（SLE）是一种累及包括外周神经系统在内的多个器官的自身免疫性疾病。我们描述了一名12岁男孩，其首发表现为进行性加重的神经症状。腿部疼痛、平衡失调和下肢无力是他入住神经科病房的原因。由于可能患有格林-巴利综合征和急性炎症性脱髓鞘性多发性神经病（AIDP），该患者开始接受静脉注射免疫球蛋白治疗。然而，治疗无明显效果，他再次出现多发性神经病的反复发作，最终被诊断为慢性炎症性脱髓鞘性多发性神经病（CIDP）。随后，他连续5天接受静脉注射甲泼尼龙冲击治疗，之后口服泼尼松龙3个月。停用糖皮质激素1个月后，他再次因相同症状入院。风湿学检查发现存在白细胞减少、溶血性贫血、血尿、蛋白尿、抗核抗体阳性和双链DNA抗体阳性。根据美国风湿病学会和系统性红斑狼疮国际协作临床中心的SLE分类标准，该患者最终被诊断为SLE。最终，他接受了甲泼尼龙和环磷酰胺冲击治疗，以及口服羟氯喹和泼尼松龙治疗。他的神经和身体症状得到改善，几个月后神经功能完全恢复。SLE和AIDP/CIDP是不同的疾病，但AIDP/CIDP可以是SLE神经表现的一部分。尽管AIDP/CIDP与SLE之间的关联非常罕见，尤其是作为SLE的首发表现，但应尽早识别以便进行快速恰当的治疗。
