以狼疮肠系膜血管炎起病的儿童系统性红斑狼疮1例报告

doi:10.12372/jcp.2022.22e0734

摘要/Abstract

摘要：

狼疮肠系膜血管炎（LMV）作为儿童系统性红斑狼疮（SLE）首发起病的情况非常罕见，临床表现为腹痛、呕吐、腹泻等非特异性症状，极易误诊。患儿，男，13岁，因腹痛伴呕吐2天就诊。入院体检示全腹肌紧张，肠鸣音减弱。辅助检查血小板30×10⁹/L，抗核抗体1:320阳性，抗心磷脂抗体IgM阳性，C3和C4降低，Coomb's试验弱阳性，24小时尿蛋白1.18 g；腹部CT示小肠肠壁明显增厚、水肿（靶征）。明确诊断为SLE、LMV，予甲基泼尼松龙60 mg及羟氯喹治疗，腹痛好转，但血小板进行性下降，先后予环孢素、甲基泼尼松龙冲击治疗血小板仍下降，予利妥昔单抗治疗后血小板逐渐升高，后续予贝利尤单抗治疗，病情平稳后出院。LMV可为SLE首发表现，腹部CT对LMV确诊有重要价值。糖皮质激素可有效减轻病情，但LMV易复发，环磷酰胺冲击及利妥昔单抗治疗可能对复发患者效果好。

关键词: 系统性红斑狼疮, 狼疮肠系膜血管炎, 狼疮肠炎, 儿童

Abstract:

Lupus mesenteric vasculitis (LMV) as the first onset of systemic lupus erythematosus (SLE) in children is very rare. The clinical manifestations are abdominal pain, vomiting, diarrhea and other non-specific symptoms, which are easy to be misdiagnosed. A 13-year-old boy was admitted to Shanghai Children's Hospital who presented with abdominal pain and vomiting for 2 days. Physical examination on admission found that the child's abdominal muscles were tense and bowel sounds were weakened. Laboratory tests showed platelets of 30×10⁹/L, positive antinuclear antibody (1:320), positive anticardiolipin antibody IgM, decreased complement C3 and C4, weakly positive Coomb's test, and 24-hour urinary protein of 1.18 g. Abdominal CT showed significantly thickened and edema intestinal wall. After the patient was diagnosed with SLE and LMV, he was treated with 60 mg of methylprednisolone and hydroxychloroquine. The abdominal pain improved, but the platelet gradually decreased. After high-dose methylprednisolone and cyclosporine pulse therapy, platelet was still decreasing. After rituximab treatment, the patient's platelets gradually increased, and he was subsequently treated with belimumab. He was discharged after his condition stabilized. LMV can be the first manifestation of SLE. Abdominal CT has an important value in the diagnosis of LMV. Glucocorticoids can effectively alleviate the disease, but LMV is prone to relapse. Cyclophosphamide pulse therapy and rituximab may be effective in patients with relapse.

Key words: systemic lupus erythematosus, lupus mesenteric vasculitis, lupus enteritis, child

刘园园, 匡新宇, 康郁林, 孙利文, 胡玉杰, 黄文彦. 以狼疮肠系膜血管炎起病的儿童系统性红斑狼疮1例报告[J]. 临床儿科杂志, 2022, 40(12): 944-949.

LIU Yuanyuan, KUANG Xinyu, KANG Yulin, SUN Liwen, HU Yujie, HUANG Wenyan. Systemic lupus erythematosus in children with onset of lupus mesenteric vasculitis: a case report[J]. Journal of Clinical Pediatrics, 2022, 40(12): 944-949.

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时间	ANA	C3/g·L^-1	C4/g·L^-1	尿蛋白定量/g	D-二聚体/mg·L^-1	血沉/mm·h^-1
6.25	1:320+颗粒型	0.61	0.03	/	30.28	15
7.03	/	0.76	0.07	1.18	7.02	/
7.13	1:100+颗粒型	0.96	0.05	/	5.67	6
7.19	/	1.07	0.08	0.23	3.62	5
7.23	/	1.24	0.08	/	3.27	18

时间	临床表现及检查情况	诊断	治疗
2021.6.21-2021.6.22 （外院）	腹痛、呕吐；血小板41×10⁹/L，腹部超声示右上腹局部肠壁水肿、增厚；腹部CT示肠系膜上静脉栓塞?小肠肠管坏死?	腹痛待查，血小板减少	禁食补液、抑酸、抗感染
2021.6.23 （本院）	腹痛、呕吐；血小板30×10⁹/L；腹部CT示小肠肠壁明显增厚、水肿（靶征），腹腔积液，考虑炎性病变伴小肠梗阻	腹痛待查，肠梗阻，血小板减少	急诊腹腔镜探查术，腹腔引流
2021.6.24-2021.6.28 （本院）	腹痛；血小板36×10⁹/L，24小时尿蛋白定量1.18 g，ANA 1:320阳性，抗心磷脂抗体IgM阳性，Coomb's试验弱阳性，C3 0.61 g/L，C4 0.03 g/L；胸部CT示两侧胸腔积液	腹痛待查，SLE?血小板减少，肠梗阻术后	甲泼尼龙、丙种球蛋白、白蛋白、抗感染
2021.6.28-2021.7.27 （本院）	腹痛逐渐消失；SLEDAI 2000评分11分，出院前降至5分	SLE、LMV	甲泼尼龙、羟氯喹、环孢素、利妥昔单抗、第1次贝利尤单抗
2021.8.9 （本院）	无症状；SLEDAI 2000评分2分	SLE、LMV	泼尼松60 mg，羟氯喹300 mg，环孢素25mg Q12h，第2次贝利尤单抗
2022.7.25 （本院）	无症状；SLEDAI 2000评分0分	SLE、LMV	泼尼松5 mg，羟氯喹300 mg，环孢素25mg Q12h，第15次贝利尤单抗

病例	年龄/岁	血常规			部分自身抗体			腹部CT	随访时长/月	复发
病例	年龄/岁	白细胞 ×10⁹/L	血红蛋白 /g·L^-1	血小板×10⁹/L	抗 Smith	抗 RNP	抗ds- DNA	腹部CT	随访时长/月	次数	距首次发病时间
1^[4]	14	正常	正常	正常	+	+	-	靶征、梳状征	34	1	2年
2^[5]	9	7.08	118	377	-	+	+	靶征	8	1	半月
3^[5]	14	1.63	115	166	-	+	+	靶征	6	0	无复发
4^[5]	12	7.59	62	320	-	±	+	梳状征	3	0	无复发
5^[6]	15	2.5	91	102	不详	不详	+	未查	12	0	无复发
6^[7]	12	3.4	101	285	-	不详	+	靶征、肠系膜脂肪衰减	不详	0	无复发
7^[8]	15	正常	80	110	-	-	+	靶征、梳状征	30	0	无复发
8^[8]	13	正常	139	187	-	-	+	靶征、梳状征	不详	多次	不详
9^[8]	15	下降	118	197	+	+	+	靶征、梳状征	不详	0	无复发
10^[9]	9	正常	正常	正常	-	不详	-	靶征、梳状征	24	3	2月、12月、14月