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

IF 1 Q3 MEDICINE, GENERAL & INTERNAL American Journal of Case Reports Pub Date : 2024-12-24 DOI:10.12659/AJCR.944829
Axelle Amiel, Théo Van Gucht, Marion Bolliet, Lucie Aussenac-Belle, Patrice David, Laurianne Plastaras, Martin Martinot
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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.

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选择性IgA缺乏和无菌性肝脓肿作为年轻女性克罗恩病的初始指标:一个案例研究
背景:年轻女性肝脏病变可导致多种诊断假设,主要是感染和肿瘤。克罗恩病(CD)很少被临床医生引起,但据报道与肝损害有关,特别是弥漫性肉芽肿和无菌性脓肿。IgA缺乏与乳糜泻或炎症性肠病相关,包括乳糜泻。在本报告中,我们报告了一名23岁的健康女性在检测到无菌性肝脓肿后诊断为乳糜泻,该脓肿与以前未知的选择性IgA缺乏相关。病例报告:一名23岁的年轻女性,一年前除阑尾炎外无其他病史,因持续发热2周并伴有c反应蛋白升高(142 mg/L)住院。腹部计算机断层密度仪和MRI显示一个4厘米的肝脓肿(IV段)。活检显示无菌上皮样巨细胞肉芽肿伴无干酪坏死,肉芽肿富含嗜酸性多核。此外,结肠镜检查发现结肠和回肠粘膜炎症伴局灶性溃疡,提示CD。免疫学评估导致选择性IgA缺乏症的诊断。抗肿瘤坏死因子和免疫抑制剂治疗导致肝脏病变迅速恢复和消退。结论无菌性肝脓肿应考虑CD。考虑到IgA缺乏与乳糜泻之间的关系,应评估乳糜泻患者的IgA(和IgG/IgM)水平。乳糜泻患者是否经常出现无菌性肝脓肿等特定表现与IgA缺乏有关,还需要进一步研究来证实。
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来源期刊
American Journal of Case Reports
American Journal of Case Reports Medicine-Medicine (all)
CiteScore
1.80
自引率
0.00%
发文量
599
期刊介绍: American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.
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