Rheumatology Department of Capital Institute of Pediatrics, Beijing, China.
Radiology Department of Capital Institute of Pediatrics, Beijing, China.
Clin Rheumatol. 2018 Jan;37(1):277-283. doi: 10.1007/s10067-017-3841-0. Epub 2017 Oct 10.
We reviewed three cases of systemic lupus erythematosus (SLE) in children with mesenteric vasculitis (LMV) as initial presentation and analysed their clinical characteristics to improve the understanding of this disease. Three patients with SLE were admitted to our hospital and initially presented with gastrointestinal symptoms. We retrospectively analysed their clinical data, including clinical presentations, laboratory results, images and short- and long-term treatment outcomes. (1) All three children were school-age girls. The patients were presented to our hospital with vomiting and abdominal pain as initial symptoms. The patients also had urinary symptoms, including proteinuria in three cases, ureteropelvic dilatation in two cases and hydronephrosis in one case. (2) The patients had various positive autoantibodies and a low complement level. Two of the patients had blood system involvement, and one had central nervous system symptoms. (3) All of the patients had active SLE (SLEDAI-2K score ≥ 5 points and moderate to severe degree 10-24). (4) Abdominal CT scans with contrast showed the 'target sign' of the intestinal wall in case 1, a slightly thickened intestinal wall and blurry mesentery in case 2, and the 'comb sign' of the margin mesenteric blood vessels in case 3. (5) All three patients responded promptly to steroid therapy. The patients' symptoms improved rapidly after treatment. LMV is a rare SLE complication. The lack of comprehensive understanding of LMV's clinical presentation makes it considerably challenging to diagnose. LMV is also a serious complication of SLE that is often accompanied by concurrent damage to other organs. LMV often occurs with active SLE but responds rapidly to glucocorticoid therapy. Therefore, in order to make early diagnosis and treatment, we suggest checking autoantibodies and abdominal CT scans with contrast when children present with gastrointestinal symptoms and the involvement of other organs, especially the urinary system.
我们回顾了以肠系膜血管炎（LMV）为首发表现的 3 例儿童系统性红斑狼疮（SLE），并分析了其临床特点，以提高对本病的认识。3 例 SLE 患儿均以胃肠道症状为首发表现入院，回顾性分析其临床资料，包括临床表现、实验室结果、影像学表现及近期和远期治疗转归。（1）3 例患儿均为学龄期女童，首发症状均为呕吐、腹痛，均伴有泌尿系统症状，其中蛋白尿 3 例，肾盂输尿管扩张 2 例，肾积水 1 例。（2）患儿均有多种自身抗体阳性，补体水平低下，2 例有血液系统受累，1 例有中枢神经系统受累。（3）3 例患儿均有活动的 SLE（SLEDAI-2K 评分≥5 分，中重度 10~24 分）。（4）腹部 CT 增强扫描均可见“靶征”1 例，肠壁稍增厚、系膜模糊 2 例，边缘肠系膜血管“梳征”3 例。（5）3 例患儿均对激素治疗反应迅速，治疗后症状迅速改善。LMV 是 SLE 的少见并发症，由于对其临床表现认识不足，诊断较困难。LMV 也是 SLE 的严重并发症，常合并其他脏器损害。LMV 常发生于活动期 SLE，但对糖皮质激素治疗反应迅速。因此，为了做到早期诊断和治疗，我们建议当儿童出现胃肠道症状及其他系统，尤其是泌尿系统受累时，要检查自身抗体及腹部 CT 增强扫描。
