Objective To analyze the clinical characteritics,diagnosis and treatment based on a lupus mesenteric vasculitis (LMV) case.Methods Clinical data of the patient with LMV were analyzed at rheumatology and immunology department of the capital institute of pediatrics.Wanfang database,Chinese Journal Fall-text Database (CJFD) and China wip database were searched by the keywords:"lupus enteritis,lupus mesenteric vasculitis,children from the time when those were built up to December 2017.Results A nine-year-old girl with LMV was in hospital,who presented with abdominal pain,vomiting and diarrhea over 2 months.Her diarrhea was about dozens of times a day and,at each time,the amount of stools was about 10ml to 100ml.The results of abdominal examination were abdominal swelling,total abdominal tenderness,bowel tone lower,shifting voiceless (+).In addition,lab examinations showed,ANAl:320 (granular type),dsDNA,Sm antibody (-),anti-SSA,SSB and RO-52 (3 +),blood C3,C4 reduced,mass proteinuria.Abdominal ultrasonography showed enlarged of bilateral renal pelvis,renal calices and ureter.The wall of urinary bladder was also thickening.The symptoms disappeared after CTX pulse and the oral MP and hydroxychloroquine.At 2 months (rotavirus infection),12 months (unclean food) and 14 months (no obvious cause) after the treatment,Some symptoms appeared that these were abdominal pain,diarrhea,green watery mucus and then vomiting and bile samples.Abdominal CT scan with contrast showed bowel wall thickening,dilatation of intestinal segments,enhanced "target sign"," comb sign",and dilated bilateral renal pelvis calyces and ureter.Based on those findings,she was diagnosed with LMV.After given large doses of MP and CTX pulse,her symptoms disappeared.But at the third time of recurrence,MP pules was not show obvious effect.So rituximab was administered two times.There were 6 SCI references were found and Chinese literatures could not been found.Currently,10 children LMV and 12 cases LMV in hospital have been reported in the international community.Four cases had not detail of clinical data.A summary of 6 cases with detail information were summarized.Conclusion LMV in children was rare and most of them were school-age girls.LMV might be the first manifestation of SLE,and also be in the stage of SLE completely relief.The typical clinical manifestations of the disease were acute abdominal pain,bile vomiting,and large amount of dark green water samples.Abdominal enhanced CT scan was key in making the correct diagnosis of LMV.The disease was easy to combine with the urinary tract abnormal,and the active treatment could be restored.So operation should be avoided as could as possible.The title of anti-SSA and SSB antibody was higher,which might be the predicting factor of the LMV.All the disease combined with lupus nephritis,including nephritic level of proteinuria.Timely diagnosis becomes very important for the adequate management of this rare entity.MP pulse was crucial and,if there was poor response,MP pulse should be prolonged.LMV was easy to relapse and rituximab might be one of a choice if there was therapy-resistant.%目的 探讨儿童狼疮肠系膜血管炎(LMV)的临床特点、诊断和治疗.方法 总结1例反复复发的LMV患儿的临床资料及诊治经过,并行文献复习.结果 女,9岁.因“腹痛、呕吐及腹泻2个月”就诊.患儿无明显诱因出现腹痛,逐渐加重,阵发性加剧,严重时似刀割样.随后出现呕吐及腹泻.呕吐物为胆汁样.腹泻初期为黄色水样便;之后呈墨绿色水样便,每日数十次,每次10～ 100 mL,含黏液.无皮疹、口腔溃疡及脱发等.辅助检查:大量蛋白尿,ANA 1∶320(颗粒型),抗SSA、抗SSB和抗R0-52均(+++).诊断儿童SLE、狼疮性肾炎,甲基泼尼松龙(MP)和环磷酰胺(CTX)冲击治疗后予MP和羟氯喹口服,症状消失.治疗后2、12和14个月,出现腹痛、腹泻和呕吐,腹部CT均见广泛肠壁增厚,增强后见“靶形征”和“梳子征”,双侧肾盂、肾盏扩张伴全程输尿管扩张及膀胱壁增厚,诊断LMV.第3次MP冲击效果不明显,予利妥昔单抗治疗,病情好转出院.随访2年,未再发现LMV.检索PubMed、万方数据库、中国期刊全文数据库和中国维普数据库,检索时间从建库起至2017年12月30日,共检索到6篇英文文献,明确报告10例LMV,1篇文献报告12例次LMV(未提及病例数).总结临床特征报告较为详细的6例和本文1例的临床特征.7例均为女孩,SLE和LMV诊断年龄9～16岁,均有腹痛和呕吐,腹泻和中枢神经系统受累各3例,血液系统受累(包括WBC低、贫血及PLT减少)、肾脏受累各6例;均ANA高滴度阳性,抗dsDNA升高6例,抗SSA、SSB抗体阳性5例,抗Sm抗体、ACL抗体(抗心磷脂抗体)阳性各2例,均有补体C3/C4下降;均予大剂量MP冲击治疗,6例予CTX冲击治疗,1例予利妥昔单抗;3例反复复发.结论 儿童LMV罕见,多为学龄女童,可以SLE为首发症状,亦可在SLE持续缓解状态下突然出现,典型表现为剧烈腹痛、胆汁样呕吐及排大量墨绿色水样便,确诊依靠腹部增强CT;易合并泌尿系统受累,积极治疗可以恢复;建议早期诊断后MP冲击治疗,效果欠佳者延长冲击时间;极易复发,CTX冲击对于预防复发的效果有限,难治性病例可考虑用利妥昔单抗.
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