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目的提高对以消化道症状为首发表现的儿童系统性红斑狼疮(SLE)的认识,以加强早期诊断及治疗。方法回顾性分析北京儿童医院消化病房2002年1月至2010年12月年收治的4例以消化道症状为首发表现的SLE患儿临床表现、影像学资料、相关实验室检查及治疗结果。结果 (1)男女比为1∶1,学龄期发病率高;(2)血液系统和补体多表现异常;(3)最大特点是消化道和膀胱泌尿系统内脏平滑肌同时受累,特征性表现为肠道炎症性浸润或假性肠梗阻、腹腔积液(浆膜炎),同时伴尿蛋白阳性、肾盂输尿管扩张,内壁粗糙、增厚,且为内脏损害的首发表现,其他系统受累表现轻;(4)自身抗体阳性检出率高;(5)所有患儿对激素及免疫抑制剂反应良好。结论以消化道症状为首发症状的SLE较少见,因症状不典型多容易漏诊和误诊。因此,以消化道症状起病且为主要表现而就诊患儿,如检查发现同时伴有输尿管扩张、尿蛋白等泌尿系改变者,应高度警惕SLE的可能,应及时行自身抗体、补体检查,以利早期诊治SLE,改善预后。
Objective To raise awareness of children with systemic lupus erythematosus (SLE), a first manifestation of gastrointestinal symptoms, to enhance early diagnosis and treatment. Methods The clinical manifestations, imaging data, related laboratory tests and treatment results of 4 children with SLE with digestive symptoms as the first manifestation were retrospectively analyzed in Beijing Children ’s Hospital digestive ward from January 2002 to December 2010. Results (1) male to female ratio of 1: 1, high incidence of school age; (2) the blood system and complement abnormalities; (3) the most prominent characteristic of the digestive tract and bladder urinary system visceral smooth muscle involvement, the characteristic manifestation of the intestine Inflammatory or pseudobiliary obstruction, peritoneal effusion (serositis), together with urinary protein positive, ureteropelvic dilatation, rough wall, thickening, and the first manifestation of visceral damage, other systems involved in the performance of light; ( 4) high positive rate of autoantibodies; (5) all children respond well to hormones and immunosuppressive agents. Conclusions SLE with gastrointestinal symptoms as the first symptom is rare, and it is easily missed and misdiagnosed because of the atypical symptoms. Therefore, the onset of gastrointestinal symptoms and the main manifestation of treatment of children, such as examination found accompanied by ureteral dilatation, urinary protein and other urinary tract changes should be highly alert to the possibility of SLE should be timely autoantibodies, complement testing, Eli early diagnosis and treatment of SLE, improve prognosis.