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

周期性发热、口疮性口炎、咽炎和宫颈腺炎（PFAPA）综合征是儿童最常见的周期性发热疾病。我们检查了临床结果和CD64（即Fcγ-RI在PFAPA患者多形核中性粒细胞和单核细胞上的表达）在诊断PFAPA患者中的应用，尽管CD64在PFAPA无发作期和对照组的表达水平相似，但CD64在中性粒细胞和单核细胞上的表达在发作期间急剧增加。此外，一些PFAPA患者在发作期间血清IFN-γ也升高，提示与t细胞活化有关。我们的研究结果表明，在PFAPA发作期间显著的CD64表达可作为诊断的潜在生物标志物。

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.