一个异常的免疫反应伪装成传染病
Quynh Kieu1,2, Brianna Leyden1
1Department of Pediatrics, CHOC Hospital, Orange, CA, USA.
SAGE open medical case reports
|March 5, 2026
概括
基库奇-富士门氏病 (KFD) 是一种罕见的淋巴腺炎. 这一案例突出的是一个年轻的女性发烧,淋巴腺病症和升高的IL-18,通过活检诊断并用皮质类固醇治疗.
科学领域:
- 儿科 儿科 儿科
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 基库奇-富士门氏病 (KFD) 是一种罕见的良性淋巴腺炎,病因不明,通常与免疫失调有关.
- 它主要影响年轻人和儿童,特别是亚裔女性.
研究的目的:
- 报告一名14岁的南亚女性KFD病例,该病例呈现复杂症状.
- 讨论KFD的诊断挑战和治疗方法.
主要方法:
- 使用了临床表现,实验室发现,成像 (超声波,CT),细胞因子面板 (IL-18) 和淋巴结活检.
- 通过综合评估,包括系统性炎症疾病在内的差异诊断被排除在外.
主要成果:
- 患者出现了长时间的发烧,头痛,眼睛发红,关节痛和全细胞减小.
- 活检证实KFD.上升的IL-18水平和囊性死淋巴腺炎在活检上证实KFD.
- 发烧和随后的症状对德克萨米他和静脉输入的皮质类固醇有反应.
结论:
- 诊断KFD需要一个高的怀疑指数,特别是在年轻的患者,无法解释的发烧和淋巴腺病变.
- 及时的皮质类固醇治疗可以有效地管理KFD症状,支持其免疫介导性质.
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