一种不典型的IgG4相关疾病的病例,呈现出高氨酸性,多神经病变和肝功能障碍
Hiroki Mukoyama1, Kosaku Murakami2, Hideo Onizawa3
1Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Modern rheumatology case reports
|September 26, 2023
概括
与免疫球蛋白G4相关的疾病 (Ig4-RD) 可以影响多个器官. 本案例报告强调了活检对异常Ig4-RD呈现的诊断重要性,有助于与其他疾病区分.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 胃肠病学 胃肠病学
背景情况:
- 免疫球蛋白G4相关疾病 (Ig4-RD) 是一种多系统纤维炎症疾病.
- Ig4-RD通常会影响诸如唾液腺,胰腺和胆道等器官.
- 非典型的表现可以模仿其他炎症和自身免疫性疾病.
研究的目的:
- 报告一种新的和非典型的Ig4-RD病例.
- 要突出诊断挑战在区分Ig4-RD和其他高氨酸性和多神经病症综合征.
- 为了强调组织活检在准确的Ig4-RD诊断中的关键作用.
主要方法:
- 临床检查一个72岁的女性,患有过高氨酸和升高的IgG4水平.
- 诊断工作包括实验室检测,成像 (胆管) 和淋巴结,肝脏和下下腺的活检.
- 对葡萄糖皮质类药物的治疗反应的评估.
主要成果:
- 这位患者呈现出过高氨基酸,IgG4升高,多神经病变,肝功能障碍,淋巴腺病变,眼腺扩大和珠珠胆道扩张.
- 活检证实了Ig4-RD的存在.
- 在葡萄糖皮质醇治疗后,症状显著改善.
结论:
- 这一案例说明了Ig4-RD的具有挑战性和非典型的表现.
- 活检对于区分Ig4-RD与诸如多炎的eosinophilic granulomatosis和异常性hypereosinophilic综合征等疾病至关重要.
- 及时诊断和用葡萄糖皮质激素治疗可以导致Ig4-RD的良好结果.
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