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一名患有早期IgA沉积于肠血管壁的儿童克罗恩病患者的IgA肾病和IgA血管炎。

IgA nephropathy and IgA vasculitis in a pediatric Crohn's disease patient with early IgA deposition in vascular walls of intestines.

作者信息

Tang Junqian, Wang Lan, Zhou Wei, Mao Youying, Zhang Chenxing, Shen Jiayao, Yin Minzhi, Yin Lei

机构信息

Department of Hematology/Oncology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, 1678 Dong Fang Road, Shanghai, 200127, China.

Department of Gastroenterology, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, 1678 Dong Fang Road, Shanghai, 200127, China.

出版信息

CEN Case Rep. 2025 Feb 26. doi: 10.1007/s13730-025-00970-x.

Abstract

Patients with inflammatory bowel disease may present with extraintestinal manifestations. Crohn's disease complicated with IgA nephropathy or IgA vasculitis is relatively rare. In this case, an 11-year-old girl was diagnosed with Crohn's disease and infliximab was administered. 1 year after treatment, she presented with asymptomatic but persistent microscopic hematuria. The child was diagnosed with IgA vasculitis and IgA nephropathy at the fourth year of follow-up. To explore the association between Crohn's disease and IgA associated diseases, immunostaining for IgA and GdIgA1 deposition was retrospectively conducted in intestinal biopsy tissues obtained at the time of initiation and relapse of Crohn's disease. GdIgA1 deposition in intestinal tissues was found not only at the time of relapse of Crohn's disease, but also at the beginning of Crohn's disease when patient had neither exposure to any drug nor any symptom of IgA vasculitis or IgA nephropathy. The early appearance of GdIgA1 deposition indicated that Crohn's disease played a greater role in its formation than infliximab induction and the child might be prone to IgA associated diseases. Patients with Crohn's disease, especially those who receive tumor necrosis factor-alpha inhibitors are recommended to receive regular kidney examinations.

摘要

炎症性肠病患者可能会出现肠外表现。克罗恩病合并IgA肾病或IgA血管炎相对少见。在此病例中,一名11岁女孩被诊断为克罗恩病并接受了英夫利昔单抗治疗。治疗1年后,她出现无症状但持续存在的镜下血尿。在随访的第四年,该患儿被诊断为IgA血管炎和IgA肾病。为了探究克罗恩病与IgA相关疾病之间的关联,对克罗恩病初发和复发时获取的肠道活检组织进行了IgA和GdIgA1沉积的免疫染色回顾性研究。结果发现,不仅在克罗恩病复发时肠道组织中有GdIgA1沉积,而且在克罗恩病初发时,即患者既未接触任何药物且无IgA血管炎或IgA肾病任何症状时,肠道组织中就有GdIgA1沉积。GdIgA1沉积的早期出现表明,克罗恩病在其形成过程中比英夫利昔单抗诱导发挥了更大作用,且该患儿可能易患IgA相关疾病。建议克罗恩病患者,尤其是那些接受肿瘤坏死因子-α抑制剂治疗的患者定期进行肾脏检查。

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