Abstract
PFAPA- Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical
Adenitis syndrome are the most common autoinflammatory syndromes in children. In
this chapter, the characteristic manifestation and clinical criteria of PFAPA, which
remain the basis of diagnosis, are presented. The therapeutic options and prognosis are
discussed in detail.
Keywords: Adenitis, Anakinra, Aphthous stomatitis, Autoinflammation, Colchicine, Interleukin-1, Interleukin-1 blockers, Marshall syndrome, Periodic fevers, PFAPA, Pharyngitis, Tonsillectomy, Tonsillotomy.
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