疾病发病时的儿童混合结合组织疾病:来自系统审查的证据
Alberto Terminiello1, Edoardo Marrani2, Ilaria Pagnini2
1Department of Health Sciences, University of Florence, Florence, Italy.
Autoimmunity reviews
|January 8, 2024
概括
儿童混合结合组织疾病 (cMCTD) 呈现出各种症状,使得诊断具有挑战性. 本综述强调了早期临床关键特征,以帮助制定更好的诊断标准,用于这种罕见的儿科疾病.
科学领域:
- 儿科风湿病学 儿科风湿病学
- 自免疫性疾病 自免疫性疾病
- 结合组织疾病 结合组织疾病
背景情况:
- 儿童混合结合组织疾病 (cMCTD) 是一种罕见的儿科自身免疫性疾病.
- 它表现出系统性红斑狼,多性肌肉炎/皮肤性肌肉炎,青少年异常性关节炎和系统性硬化症的重叠特征.
- 对cMCTD的诊断仍然具有挑战性,因为它的罕见性和异质呈现.
研究的目的:
- 系统地审查和识别cMCTD发病时的临床特征.
- 识别目前未包括在现有cMCTD诊断标准中的表现.
- 为改善cMCTD的诊断方法的开发提供信息.
主要方法:
- 按照PRISMA 2020指南进行了一次系统的文献审查.
- 搜索的数据库包括MEDLINE (PubMed) 和EMBASE.
- 包括的研究分析了诊断为MCTD的儿科患者呈现的迹象和症状.
主要成果:
- 分析了39篇文章,涉及215名儿科患者,82.5%是女性.
- 最常见的表现特征包括雷诺现象 (69.7%),关节炎 (60.9%) 和肌肉参与 (53.5%).
- 其他显著的表现包括皮肤病征兆 (39.5%) 和手指/手部胀 (29.3%).
结论:
- cMCTD的临床表现非常异质.
- 目前对cMCTD的诊断标准可能不包括早期表现的全部范围.
- 需要进一步的研究来开发更准确和更早的cMCTD诊断方法.
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