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选择性IgA缺乏症和无菌性肝脓肿作为一名年轻女性克罗恩病的初始指标:病例研究

Selective IgA Deficiency and Aseptic Liver Abscess as Initial Indicators of Crohn's Disease in a Young Woman: A Case Study.

作者信息

Amiel Axelle, Van Gucht Théo, Bolliet Marion, Aussenac-Belle Lucie, David Patrice, Plastaras Laurianne, Martinot Martin

机构信息

Department of Infectious Diseases, Hôpitaux Civils de Colmar, Colmar, France.

Department of Hepato-Gastroenterology, Hôpitaux Civils de Colmar, Colmar, French Guiana.

出版信息

Am J Case Rep. 2024 Dec 24;25:e944829. doi: 10.12659/AJCR.944829.

Abstract

BACKGROUND Hepatic lesion in a young woman can lead to multiple diagnostic hypotheses, mainly infection and tumor. Crohn's disease (CD) is hardly evoked by clinicians but is reportedly associated with liver damage, especially diffuse granulomas and aseptic abscess. IgA deficiency has been associated with celiac disease or inflammatory bowel disease, including CD. In this report, we present the diagnosis of CD in a fit 23-year-old woman following detection of aseptic liver abscess associated with a previously unknown selective IgA deficiency. CASE REPORT A young 23-year-old woman with no previous medical history other than appendicitis 1 year ago was hospitalized with persistent fever for 2 weeks associated with C-reactive protein increase (142 mg/L). Abdominal computed tomodensitometry and MRI showed a 4-cm liver abscess (segment IV). Biopsy revealed an aseptic epithelioid gigantocellular granuloma with caseous-free necrosis and granulomas rich in eosinophilic polynuclei. Furthermore, colonoscopy detected an inflammation in the colonic and ileal mucosa, with focal ulcerations, suggestive of CD. Immunological assessment led to the diagnosis of selective IgA deficiency. Anti-TNF and immunosuppressor therapies led to a rapid recovery and regression of hepatic lesions. CONCLUSIONS CD should be considered in aseptic liver abscess cases. Considering the association between IgA deficiency and CD, IgA (and IgG/IgM) should be assessed in patients with CD. Further research is necessary to confirm if specific manifestations such as aseptic liver abscess frequently occur in patients with CD related to IgA deficiency.

摘要

背景

年轻女性的肝脏病变会引发多种诊断假设,主要是感染和肿瘤。临床医生很少会想到克罗恩病(CD),但据报道它与肝损伤有关,尤其是弥漫性肉芽肿和无菌性脓肿。IgA缺乏与乳糜泻或包括CD在内的炎症性肠病有关。在本报告中,我们介绍了一名23岁健康女性在检测到与先前未知的选择性IgA缺乏相关的无菌性肝脓肿后被诊断为CD的病例。病例报告:一名年轻的23岁女性,除1年前患过阑尾炎外无既往病史,因持续发热2周且C反应蛋白升高(142mg/L)入院。腹部计算机断层扫描和磁共振成像显示一个4厘米的肝脓肿(IV段)。活检显示为无菌性上皮样巨细胞肉芽肿,伴有无干酪样坏死和富含嗜酸性多形核细胞的肉芽肿。此外,结肠镜检查发现结肠和回肠黏膜有炎症,伴有局灶性溃疡,提示CD。免疫评估确诊为选择性IgA缺乏。抗TNF和免疫抑制剂治疗使肝脏病变迅速恢复和消退。结论:无菌性肝脓肿病例应考虑CD。鉴于IgA缺乏与CD之间的关联,CD患者应评估IgA(以及IgG/IgM)。有必要进一步研究以确认与IgA缺乏相关的CD患者是否经常出现无菌性肝脓肿等特定表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/096a/11684345/aa89917420c7/amjcaserep-25-e944829-g001.jpg

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