儿童IgG4相关疾病:临床表现,管理和诊断挑战
Silvia Taranto1, Luca Bernardo2, Angela Mauro3
1Pediatric Department, "Vittore Buzzi" Children's Hospital, 20154 Milan, Italy.
与免疫球蛋白G4相关的疾病 (IgG4-RD) 在儿童中很少见,通常呈现为局部轨道疾病. 了解其独特的儿科表现和诊断挑战对于有效管理和预防不可逆转的器官损伤至关重要.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 免疫学 免疫学 免疫学
- 眼科医生 眼科 眼科
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.
- 儿科IgG4-RD不常见,主要影响有局部表现的青少年,通常是轨道的.
研究的目的:
- 为提供儿科IgG4-RD的概述.
- 突出儿童独特的表现,诊断挑战和管理策略.
主要方法:
- 使用IMRAD方法对文献进行叙述性审查.
- 搜索了PubMed,EMBASE和科学网络直到2024年1月.
- 包括原始论文,病例报告/系列和评论,补充了两个儿科病例.
主要成果:
- 儿科IgG4-RD通常呈现为局部性疾病,通常会影响轨道,并表现出诸如proptosis和diplopia等症状.
- 成人诊断标准可能不完全适用于儿科表型.
- 这种情况是慢性,惰,并有复发性缓解性.
结论:
- 儿童IgG4-RD的早期诊断和适当的治疗对于预防渐进性纤维化和不可逆转的器官损伤至关重要.
- 需要进一步的研究来完善儿童的诊断标准和治疗方案.
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