内IgG4相关疾病:从两个病例的见解
Shaurav Khanna1, Sandeep Yadav2, C Balakrishnan3
1Clinical Fellow, Department of Rheumatology, PD Hinduja Hospital and Medical Research Centre, Mumbai, Maharashtra, India, Orcid: https://orcid.org/0009-0004-9454-040X, Corresponding Author.
The Journal of the Association of Physicians of India
|November 26, 2025
概括
影响中枢神经系统 (CNS) 的免疫球蛋白G4相关疾病 (IgG4-RD) 呈现不同. 早期诊断和用皮质类固醇和甲铁酸治疗对中枢神经系统IgG4-RD显示出有前途的结果.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
背景情况:
- 免疫球蛋白G4相关疾病 (IgG4-RD) 是一种罕见的纤维炎症疾病.
- 中枢神经系统 (CNS) 的参与很少 (2-4%),经常模仿其他疾病并导致诊断延迟.
研究的目的:
- 为了说明中枢神经系统IgG4-RD.的不同表现.
- 突出这一罕见疾病的诊断和治疗考虑因素.
主要方法:
- 病例报告1:一名29岁的男性发作,通过组织病理学诊断出持续性IgG4-RD (IgG4:IgG比率为20%),并接受皮质类固醇和甲醇治疗.
- 病例报告2:一名44岁的女性患有低体质炎和多系统干扰,诊断出可能的IgG4-RD,并接受了类固醇,甲基和德斯摩辛的治疗.
主要成果:
- 病例1显示完整的放射性解像度和治疗后的控制.
- 病例2经历了正常化的IgG4水平的临床和放射性改善.
- 这两种病例都表现出对免疫抑制疗法的反应.
结论:
- 中枢神经系统IgG4-RD表现出显著的临床异质性,从孤立的脑膜炎到多系统性脑膜炎.
- 较低的IgG4:IgG比率 (例如,20%) 在脑膜疾病中可能具有诊断意义.
- 皮质类固醇和甲醇是中枢神经系统IgG4-RD的有效一线治疗方法,需要器官特定的诊断灵活性和长期管理.
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