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

点击翻译按钮获取中文摘要

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.