PFAPA syndrome
Pronunciation: puh-FAH-pah
Also written as: PFAPA — Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis
A periodic fever syndrome characterized by recurrent episodes of fever, aphthous stomatitis, pharyngitis, and cervical adenitis in children.
Full Definition
PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) syndrome is the most common periodic fever syndrome in children, typically presenting in early childhood with highly predictable episodes of high fever lasting 3-5 days every 4-6 weeks. Between episodes, children are completely well with normal growth and development. The condition is distinguished from infectious causes by its periodicity, lack of response to antibiotics, and dramatic response to corticosteroids. Diagnosis is clinical, based on established criteria, as no specific laboratory test exists. Treatment options include corticosteroids for acute episodes or tonsillectomy for frequent, severe episodes.
Usage
Usage note: All caps for acronym; distinguish from other hereditary periodic fever syndromes.
In Context
- "The child's recurrent fevers every 4 weeks with pharyngitis and adenitis suggested PFAPA syndrome." — Clinical assessment
- "PFAPA syndrome was confirmed when the fever episode resolved within hours of prednisolone administration." — Diagnostic evaluation