Department of Pediatrics, Allergy Immunology Unit, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Department of Pediatric Surgery, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Lupus. 2021 Oct;30(12):2003-2007. doi: 10.1177/09612033211045923. Epub 2021 Oct 15.
Systemic lupus erythematosus (SLE) is a complex multisystemic autoimmune disease. Lupus enteritis (LE), one of the less commonly described manifestations of childhood SLE, presents with relatively nonspecific clinical and laboratory features. In addition, recurrent episodes of LE occurring in temporal proximity are rare in children. Presence of disease activity at other sites (which may not be seen universally) supports the diagnosis of LE in an appropriate setting. Because of its potential role to cause ischemic complications, early recognition and prompt treatment are necessary for a good outcome. Herein, we describe a child with recurrent LE with an interval of about 3 months between the first and the second episode. The first episode correlated with systemic disease activity and bowel thickening was noted on abdominal ultrasonography. This episode was successfully managed with intravenous methylprednisolone pulse therapy. Conversely, the second episode was not associated with significant clinical and laboratory evidence of disease activity at other sites and the initial abdominal ultrasonography was non-contributory. Diagnostic and therapeutic delays, hence, led to the development of fatal complications. We highlight that a high index of suspicion of LE and a timely aggressive treatment is imperative for optimal outcomes even in rare pediatric cases of recurrent LE that may have normal imaging findings initially and may not be associated with systemic lupus erythematosus disease activity index (SLEDAI).
系统性红斑狼疮(SLE)是一种复杂的多系统自身免疫性疾病。狼疮性肠炎(LE)是儿童 SLE 中较少描述的表现之一,其具有相对非特异性的临床和实验室特征。此外,儿童中很少发生在时间上接近的反复 LE 发作。在其他部位存在疾病活动(并非普遍可见)有助于在适当的情况下诊断 LE。由于其可能导致缺血性并发症,因此需要早期识别和及时治疗以获得良好的结果。在此,我们描述了一例儿童反复发作的 LE,两次发作之间的间隔约为 3 个月。第一次发作与系统性疾病活动相关,腹部超声检查显示肠壁增厚。该发作通过静脉注射甲基强的松龙脉冲治疗成功控制。相反,第二次发作与其他部位无明显的临床和实验室疾病活动证据相关,最初的腹部超声检查无明显异常。因此,诊断和治疗的延误导致了致命并发症的发生。我们强调,即使在最初影像学检查正常且可能与系统性红斑狼疮疾病活动指数(SLEDAI)无关的罕见儿童复发性 LE 病例中,也需要高度怀疑 LE,并及时进行积极治疗,以获得最佳结果。