Abstract
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.关键词
系统性红斑狼疮/狼疮肠系膜血管炎/狼疮肠炎/儿童Key words
Systemic Lupus Erythematosus/Lupus Mesenteric Vasculitis/Lupus Enteritis/Children
