PFAPA Syndrome
Pronunciation: PEE-fah-pah
Also written as: PFAPA — Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis
A childhood autoinflammatory disorder characterised by regularly recurring episodes of periodic fever accompanied by aphthous stomatitis, pharyngitis, and cervical adenitis.
Full Definition
PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) syndrome is the most prevalent periodic fever disorder in young children, typically presenting before age five with strikingly predictable fever cycles occurring every three to eight weeks. Each episode lasts three to six days and resolves completely between attacks, with normal growth and development. The aetiology remains unclear; no causative monogenic mutation has been consistently identified, though polygenic contributions are suspected. Corticosteroid administration aborts individual attacks rapidly but may shorten the inter-episode interval. The diagnosis is clinical; editors must render the acronym PFAPA in all capitals and spell it out in full on first mention. Tonsillectomy is curative in a significant proportion of cases.
Usage
Usage note: Always render in full capitals: PFAPA. Spell out at first mention. Do not confuse with other monogenic periodic fever syndromes such as FMF or TRAPS, which have identified genetic causes.
In Context
- "PFAPA syndrome was diagnosed in a four-year-old with monthly fever cycles reliably accompanied by aphthous ulcers and cervical lymphadenopathy." — Pediatric immunology outpatient letter
- "The author wrote 'Pfapa' in mixed case; the editor corrected this to 'PFAPA' in accordance with standard nomenclature." — Manuscript proofreading note