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Periodic fever syndrome in children

K T Thomas1, H M Feder, A R Lawton

  • 1School of Medicine, Vanderbilt University, Nashville, Tennessee, USA.

The Journal of Pediatrics
|July 7, 1999
PubMed

Insights

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children typically resolves without long-term issues. While some cases spontaneously remit, others persist but become less frequent over time.

Area of Science:

  • Pediatrics
  • Immunology
  • Rheumatology

Background:

  • Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a common autoinflammatory disorder in children.
  • Characterized by recurrent episodes of fever, oral ulcers, pharyngitis, and cervical lymphadenopathy.

Purpose of the Study:

  • To delineate the clinical presentation, disease course, treatment efficacy, and long-term outcomes of PFAPA syndrome.
  • To identify diagnostic criteria and management strategies for PFAPA.

Main Methods:

  • A registry of 94 patients with PFAPA was established over a 10-year period.
  • Data collection included registry forms and medical records.
  • Follow-up telephone calls assessed episode persistence and sequelae.

Main Results:

  • PFAPA episodes averaged 4.8 days, recurring every 28 days with high fevers.
  • Of 83 children followed, 34 experienced spontaneous resolution.
  • Remaining patients had less frequent episodes; no long-term sequelae were observed.
  • Glucocorticoids were effective; tonsillectomy and cimetidine showed some success.

Conclusions:

  • PFAPA syndrome is a frequent cause of periodic fever in pediatric populations.
  • The syndrome may resolve spontaneously or persist with decreasing frequency.
  • PFAPA does not lead to long-term health complications.
  • Diagnosis is facilitated by recurrent fever coupled with aphthous stomatitis, pharyngitis, or cervical adenitis.
Abstract

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