PFAPA syndrome in children

Area of Science:

• Pediatrics
• Immunology
• Rheumatology

Background:

• Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, also known as Marshall syndrome, is a common autoinflammatory disorder.
• It is characterized by recurrent episodes of fever, aphthous ulcers, pharyngitis, and cervical adenitis.
• PFAPA syndrome primarily affects young children, usually under age 5, but can occur in adults.

Purpose of the Study:

• To provide a comprehensive overview of PFAPA syndrome.
• To discuss its clinical presentation, diagnosis, and management.
• To highlight its typical self-limiting course in pediatric patients.

Main Methods:

• This review synthesizes current knowledge on PFAPA syndrome.
• It includes information on diagnostic criteria, laboratory findings, and treatment approaches.
• Emphasis is placed on its nature as a diagnosis of exclusion.

Main Results:

• PFAPA syndrome presents with cyclical fevers and associated symptoms like oral ulcers and sore throat.
• Laboratory tests are generally unremarkable, apart from elevated acute-phase reactants (e.g., C-reactive protein).
• Systemic steroids are the primary treatment to manage inflammatory flares.

Conclusions:

• PFAPA syndrome is a benign, self-limited autoinflammatory condition.
• It typically resolves spontaneously in children between ages 7 and 8.
• While generally resolving on its own, supportive care and anti-inflammatory treatments manage acute episodes.