Diagnosis and treatment of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome

周期性发热、口疮性口炎、咽炎、颈腺炎综合征的诊治

• 批准号: 22591155
• 负责人: AGEMATSU Kazunaga
• 金额: $ 2.83万
• 依托单位: Shinshu University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2010
• 资助国家: 日本
• 起止时间: 2010 至 2012
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-22591155/
• 关键词: 周期性発熱; 自己炎症疾患; CD64; autoinflammatory disease; 炎症; 発熱; 治療; 周期性発熱; 自己炎症疾患; CD64; autoinflammatory disease; 炎症; 発熱; 治療

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever disease in children. We examined clinical findings and the utility of CD64, namely Fcγ-RI expression on polymorphonuclear neutrophils and monocytes in diagnosing patients with PFAPA, Although CD64 was expressed at a similar level in the attack-free period of PFAPA and controls, CD64 expressions on both neutrophils and monocytes was dramatically increased during attacks. In addition, serum IFN-γ also increased in some PFAPA patients during flares, suggesting the involvement of T-cell activation. Our findings demonstrate that remarkable CD64 expression during PFAPA flares serves as a potential biomarker for the diagnosis.

周期性热，寒冷的口腔炎，咽炎和宫颈腺炎（PFAPA）的综合征是儿童中最常见的周期性发烧疾病。我们检查了CD64的临床发现和实用性，即在PFAPA诊断患者中对聚甲核中性粒细胞和单核细胞的Fcγ-RI表达，尽管CD64在PFAPA和对照中的无攻击时期表达了相似的CD64，但在两个中性粒细胞和单粒细胞的cd64表达情况下，cd64表达式增强了。此外，在耀斑期间，一些PFAPA患者的血清IFN-γ也增加了，这表明T细胞激活参与。我们的发现表明，PFAPA耀斑期间出色的CD64表达是诊断的潜在生物标志物。