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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
Summary
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.