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PFAPA syndrome: with regard to a case
S Hernández-Bou1, M Giner, A M Plaza
1Sección Inmunoalergia. Unidad Integrada de Pediatría Hospital Clínic-Hospital Sant Joan de Déu. Universidad de Barcelona, Spain.
Allergologia Et Immunopathologia
|August 2, 2003
Summary
PFAPA syndrome, a cause of periodic fever in children, presents with fever, sore throat, and swollen glands. Prompt recognition and treatment with corticosteroids can resolve symptoms and reduce parental anxiety.
Area of Science:
- Pediatric Rheumatology
- Immunology
Background:
- PFAPA syndrome (Periodic Fever, Aphtas, Pharyngitis, and cervical Adenopathies) is a common cause of recurrent fever in children.
- Its exact cause remains unknown, and diagnosis relies on clinical presentation.
- Laboratory findings are typically non-specific.
Observation:
- A case study of a 10-year-old patient diagnosed with PFAPA syndrome after a 3.5-year history of characteristic symptoms.
- Diagnostic criteria were met, and other similar conditions were excluded.
- The patient experienced recurrent episodes of high fever, pharyngitis, and cervical adenitis.
Findings:
- PFAPA syndrome is characterized by periodic episodes of high fever, pharyngitis, and cervical adenitis.
- A rapid and effective response to one or two doses of oral prednisone (1 mg/kg) is a hallmark of the condition.
- The syndrome is benign and typically resolves within four years without lasting effects.
Implications:
- Early identification of PFAPA syndrome is crucial for appropriate management.
- Recognizing this condition can improve diagnostic and therapeutic approaches, reducing patient and family anxiety.
- Timely diagnosis and treatment with corticosteroids can significantly alleviate symptoms and prevent unnecessary investigations.