青少年发病系统性硬化症的临床和流行病学特征来自日本全国调查
Utako Kaneko1, Takako Miyamae2, Yasuhito Hamaguchi3
1U. Kaneko, MD, PhD, Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
The Journal of rheumatology
|September 15, 2025
概括
这项研究显示,日本的青少年发病系统性硬化症 (jSSc) 具有高抗毒素酶I抗体 (ATA) 阳性率. 然而,日本jSSc患者的主要器官参与反映了西方队列的发现.
科学领域:
- 类风湿病学 类风湿病学
- 儿童自身免疫性疾病 儿童自身免疫性疾病
- 流行病学 流行病学
背景情况:
- 青少年发作的全身性硬化症 (jSSc) 是一种罕见的自身免疫性疾病,影响儿童.
- 了解jSSc的临床和流行病学特征对于诊断和管理至关重要.
- 关于日本的jSSc患病率和特征的数据有限,需要进行全面的国家调查.
研究的目的:
- 确定日本青少年发病系统性硬化症 (jSSc) 的临床和流行病学特征.
- 识别JSSc表现中的潜在种族和世代差异.
- 估计日本儿科人口中jSSc的发病率和流行率.
主要方法:
- 在2016年1月至2020年12月期间,对日本机构进行了一项全国性调查.
- 估计了jSSc患者的数量,并计算了儿童 (<18岁) 的年度发病率.
- 分析了基于疾病亚型,自身抗体状态和年龄的临床特征,与先前的研究进行比较.
主要成果:
- 在日本,每年估计的jSSc发病率为每10万名儿童0.98-1.59.
- 在130个分析的病例中,65.4%患有扩散性皮肤SSc (dcSSc),中位数发病年龄为11岁.
- 观察到高抗毒素酶I抗体 (ATA) 阳性 (62.4%);间歇性肺病发生在40.8%的患者中.
结论:
- 这项研究是日本最大规模的全国性调查,详细介绍了根据自身抗体和亚型的jSSc特征.
- 日本的jSSc表现出特别高的ATA阳性率.
- 在日本jSSc患者中,主要器官参与的频率与西方队列相似.
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