儿科IgG4相关疾病表现为复发性多叶膜溢出
Umaserma Bharathi1, Padmasani Venkat Ramanan2, Shwetha Amuthan1
1Paediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
BMJ case reports
|July 1, 2025
概括
本案例研究突出了IgG4相关的疾病 (IgG4-RD) 在一个青少年呈现的复发性多流液. 诊断需要先进的成像和活检,证实IgG4-RD是儿科肺部疾病的罕见原因.
科学领域:
- 儿科肺病学 儿科肺病学
- 类风湿病学 类风湿病学
- 病理学 病理学 病理学
背景情况:
- 青少年的复发性多流往往归因于结核病或恶性瘤.
- 对常见病因的初步评估可能是不确定的,需要更广泛的差异诊断.
研究的目的:
- 报告一种罕见的IgG4相关疾病病例,呈现为青少年的复发性多流液.
- 强调诊断方面的挑战以及在耐火病例中考虑IgG4-RD的重要性.
主要方法:
- 呼吸困难,厌食症和减肥的临床表现.
- 诊断工作包括肺部液体分析,结核病检测和PET-CT扫描.
- 胸腔镜引导的肺部活检用于组织病理学检查.
主要成果:
- 对结核病和恶性瘤的初始检查结果为负.
- PET-CT显示了右侧的水肺胸部,软组织变厚.
- 活检证实了IgG4相关的疾病,具有特征性发现:史托里形纤维化,淋巴细胞透,消灭性炎和>30%的IgG4阳性血细胞.
结论:
- 与IgG4相关的疾病是一种罕见但重要的考虑因素,对于未成年人的肺溢液没有对标准治疗的反应.
- 多模式诊断方法,包括先进的成像和向活检,对于准确的诊断至关重要.
- 用类固醇和mycophenolate mofetil等免疫抑制剂及时治疗可以带来有利的结果.
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