Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Abstract

Aim: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of a periodic fever in childhood. The exact pathogenesis and the aetiology of PFAPA are still unknown.

Methods: We conducted a non-systematic review of published articles about PFAPA syndrome and summarised the evidence for diagnostic criteria and treatment options for PFAPA.

Results: The first proposed diagnostic criteria for PFAPA, in addition to periodic fever, included aphthous stomatitis, pharyngitis or cervical lymphadenitis in children younger than five years at the beginning of the symptoms. C-reactive protein (CRP) levels and leucocyte counts increase in most patients during episodes. Recent research reveals that tonsillectomy provides an immediate and long-lasting cure for PFAPA, even in the absence of classic criteria of aphthous stomatitis, pharyngitis or cervical adenitis and in children older than five years.

Conclusion: We suggest that PFAPA can be diagnosed in children with at least five regularly occurring fever episodes without any other explanation, even in the absence of aphthous stomatitis, pharyngitis or cervical lymphadenitis and also in children older than five years.

Keywords: Aphthas; Autoinflammatory disease; Recurrent fever; Regularity; Tonsillectomy.