儿童系统性红斑狼疮合并脑后部可逆性脑病综合征4例并文献复习

Report of four cases of posterior reversible encephalopathy syndrome in children with systemic lupus erythematosus and literature review

目的分析儿童系统性红斑狼疮（SLE）合并脑后部可逆性脑病综合征（PRES）的临床特征,提高对本病的认识。方法报告北京协和医院诊断的4例儿童SLE合并PRES的临床资料,在Pub Med数据库检索相关病例行文献复习,分析儿童SLE合并PRES的临床表现、影像学检查、治疗及预后情况。结果 14例SLE患儿均伴有狼疮性肾炎,从确诊SLE至出现PRES的时间1-63个月,出现PRES时1例正在接受甲泼尼龙及环磷酰胺（CTX）冲击治疗。PRES均以惊厥、头痛起病,并伴血压升高。2Pub Med数据库检索到11例SLE合并PRES的儿童病例,结合本文报道的4例,15例进入分析。女性14例。年龄最小8岁。从确诊SLE至出现PRES的间隔中位时间为6个月（1个月至8年）。15例出现PRES时均有惊厥发作,10例伴头痛,7例呕吐,9例意识丧失,7例视力障碍。15例均有血压升高。12例有狼疮性肾炎。治疗SLE予甲泼尼龙或CTX冲击治疗分别为4和3例,予羟氯喹2例,予环孢素和利妥昔单抗各1例,PRES分别发生在免疫抑制剂治疗后的2 d至4年。15例行头颅MRI检查示大脑后循环皮质下白质受累为主。12例予降血压治疗,10例予抗惊厥药物短期治疗。9例SLE处于活动期,继予糖皮质激素和免疫抑制剂治疗;6例非活动期的SLE患儿减停糖皮质激素及免疫抑制剂。15例神经系统症状均恢复,随访均未遗留神经系统后遗症。9例复查头颅MRI示颅内病变完全或基本消失。结论儿童SLE合并PRES主要表现为惊厥、头痛、意识障碍和视觉障碍。对于有狼疮性肾炎的SLE患儿血压升高时,尤其同时予大剂量糖皮质激素或CTX等免疫抑制剂治疗时,应警惕PRES的发生。早期诊断和治疗PRES预后较好。

Objective To study the clinical profile of the posterior reversible encephalopathy syndrome（ PRES） in children with systemic lupus erythematosus（ SLE）. Methods The analysis was based on 4 PRES patients with SLE admitted to our department（ PUMCH） and 11 cases retrieved from Pub Med to analyze the clinical manifestation,PRES features,laboratory abnormalities,treatment and outcomes. Results Firstly,4 cases were diagnosed in our hospital,disease duration of SLE ranged from 1 month to 63 months. Nephritis was found in all these patients,and methylprednisolone and cyclophosphamide pulse therapy was administered in one patient. Four children with PRES presented seizures,headache and hypertension. Magnetic resonance showed predominantly posterior distribution involved. Secondly,11 cases were retrieved from Pub Med,together with our 4 cases,15 cases（ 14 girls）were analyzed. The minimum age was 8 years. The average duration between SLE diagnosis and primary onset of PRES was 6months（ ranged from 1 month to 6 years）. Clinical manifestations of PRES in children with SLE were seizures（ all of 15 cases,100. 0%）,headache（ 10 /15,66. 7%）,vomiting（ 7,46. 7%）,loss of consciousness（ 9,60. 0%） and vision loss（ 7 /15,46.7%）. Hypertension was seen in all 15 patients; 12 had nephritis; 4 were treated with methylprednisolone pulse therapy; 3 were treated with cyclophosphamide pulse therapy; 2 were treated with hydroxychloroquine; 1 was treated with cyclosporine A; 1 was treated with Rituximab. PRES occurred 2 days to 4years after immunosuppressive drugs therapy. All patients underwent MRI and the white matter of posterior region was mainly involved,and involvement in areas such as parietal lobes,brain stem and cerebellum was also found. 12 cases were treated with antihypertensive drugs. 10 cases were treated with anticonvulsant drugs in short duration. Glucocorticoid and immunosuppressive drug were used in 9 patients with active lupus,but they were withheld or reduced if the child was not accompanied by active lupus. All patients improved without neurological deficit. Follow up MRI showed marked improvement. Conclusion PRES in children with SLE presented seizures,headache,vomiting,loss of consciousness and vision loss. SLE patients with lupus nephritis,hypertension and use of immunosuppressive drug could precede the occurrence of PRES.Brain MRI is important for diagnosis of PRES. Early diagnosis,prompt control of blood pressure and seizure and appropriate use of glucocorticoid and immunosuppressive drug are keys to successful management of PRES.