Abdominal manifestations in childhood-onset systemic lupus erythematosus
Abdominal manifestations in childhood-onset systemic lupus erythematosus
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DOI：
10.1136/ard.2005.050070
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发表时间：
2007-02-01
影响因子：
27.4
通讯作者：
Bader-Meunier, B.
中科院分区：
文献类型：
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作者：
Richer, O.;Ulinski, T.;Bader-Meunier, B.
背景：儿童期发病的红斑狼疮是一种病因不明的罕见疾病。目的：描述系统性红斑狼疮SLE发病及随访时胃肠道表现的频率，并探讨这些表现的具体原因。方法：回顾法国儿科肾病、血液学和风湿病中心201例儿童期SLE患者的病历，并总结其胃肠道表现。结果：39例（19%）患儿胃肠道受累。初始胃肠道表现时的中位（范围）年龄为11.3（4.5-16）岁。32%的患者在诊断后1个月内出现或出现胃肠道症状。腹痛是最常见的症状，34例（87%）患者出现腹痛。主要与狼疮累及有关，尤其是腹水（n = 14）和胰腺炎（n = 12），较少与治疗引起的事件（n = 1）或感染（n = 1）有关，与SLE无关的事件从未发生过。三名患有手术腹部的儿童在SLE诊断前接受了开腹手术，最终诊断为狼疮性腹膜炎和狼疮性无结石性胆囊炎。除2例手术腹部患者外，其余患者C反应蛋白值均< 40 mg/l。腹部超声检查和计算机断层扫描异常的比例分别为58%和83%。在8例患者中，皮质类固醇与静脉注射环磷酰胺相关，导致31例治疗患者中30例胃肠道受累完全缓解。结论：胃肠道受累在SLE患儿中很常见，主要是由于原发性狼疮受累。在感染性疾病后出现腹痛的狼疮患儿应及时考虑皮质类固醇治疗；排除了治疗的副作用和肠道穿孔。
Background: Childhood-onset lupus erythematosus is a rare disorder of unknown origin.Objectives: To describe the frequency of gastrointestinal manifestations at presentation of systemic lupus erythematosus SLE and at follow-up, and discuss the specific causes of these manifestations.Methods: Medical records of 201 patients with childhood-onset SLE followed up in French paediatric nephrological, haematological and rheumatological centres were reviewed and abstracted for gastrointestinal manifestations.Results: Gastrointestinal involvement was recorded in 39 (19%) children. The median (range) age at the time of initial gastrointestinal manifestations was 11.3 (4.5-16) years. Gastrointestinal symptoms were present at or occurred within 1 month after diagnosis in 32% patients. Abdominal pain was the most frequent symptom, present in 34 (87%) patients. It was mostly related to lupus involvement, especially ascites (n = 14) and pancreatitis (n = 12), more rarely to treatment-induced events (n = 1) or infection (n = 1) and never to events unrelated to SLE. Three children with surgical abdomen underwent a laparotomy before SLE was diagnosed, with a final diagnosis of lupus peritonitis and lupus acalculous cholecystitis. C reactive protein values were < 40 mg/l in all but two patients who had surgical abdomen. Abdominal ultrasonography and computed tomography scans were abnormal in 58% and 83% of the evaluated patients, respectively. Corticosteroids, associated with intravenous cyclophospamide in eight patients, led to complete remission of gastrointestinal involvement in 30 of 31 treated patients.Conclusion: Gastrointestinal involvement is common in children with SLE, and is mainly due to primary lupus involvement. Corticoidsteroid treatment should be promptly considered in children with lupus presenting with abdominal pain after infectious disease; side effects of treatment and intestinal perforation have been excluded.