Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome / 国际儿科学杂志

ABSTRACT

Periodic fever, aphthous stomatitis, pharyngitis and adenitis(PFAPA)syndrome which is generally considered to be an autoinflammatory disease with multiple etiologies and heterogeneous inheritance, is the most common cause of periodic fever in childhood.So far, many possible causative factors have been explored, including infectious agents, immunologic mechanisms and genetic predisposition, but the exact etiology remains unclear.At the same time, due to the lack of standardized diagnosis and treatment standards, many children received unnecessary examinations and unreasonable treatment.This review aims to analyze PFAPA syndrome in detail in terms of clinical characteristics, pathogenesis, diagnosis, differential diagnosis and treatment in order to establish the more specific diagnostic criteria and the more standardized treatment to enable children with PFAPA to receive timely diagnosis and appropriate treatment.