[Periodic fever in children: keep in mind the PFAPA syndrome]

Area of Science:

• Pediatrics
• Immunology
• Genetics

Context:

• Autoinflammatory diseases present as recurrent fevers in children, often lacking a clear cause.
• Familial Mediterranean Fever is a genetic autoinflammatory disease requiring chronic management to prevent severe complications.
• PFAPA syndrome is the most common cause of recurrent fever in children, diagnosed via nonspecific criteria.

Purpose:

• To highlight the importance of considering autoinflammatory diseases in the differential diagnosis of recurrent fevers in childhood.
• To discuss the characteristics, diagnosis, and controversial treatment options for PFAPA syndrome.

Summary:

• Autoinflammatory diseases are characterized by recurrent inflammatory episodes without apparent triggers.
• PFAPA syndrome, a frequent cause of recurrent childhood fever, is diagnosed using nonspecific criteria.
• While treatment for PFAPA syndrome remains debated, a single dose of Prednisone can halt flares, and tonsillectomy often leads to remission.

Impact:

• Emphasizes the need for timely diagnosis of autoinflammatory conditions to guide appropriate management.
• Suggests effective interventions for PFAPA syndrome, including pharmacological and surgical options.
• Aims to improve patient outcomes by informing clinical practice regarding recurrent fevers in pediatric populations.