Diagnosis of PFAPA syndrome applied to a cohort of 17 adults with unexplained recurrent fevers

CER5010
2012 Vol.30, N°2
PI 0269, PF 0271
Brief Papers

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PMID: 22325152 [PubMed]

Received: 18/08/2011
Accepted : 26/10/2011
In Press: 13/04/2012
Published: 13/04/2012

Abstract

BACKGROUND: The pathophysiology of PFAPA syndrome, mainly characterised by regularly recurring periodic fevers associated with aphthous stomatitis, pharyngitis and/or lymphadenitis, and mostly occurring in the paediatric setting, resembles an acquired autoinflammatory disease. The description of PFAPA syndrome in adult patients is largely increasing. OBJECTIVES:
To recognise PFAPA syndrome in a group of adult patients evaluated for recurrent fevers in our Rheumatology Unit.
METHODS:
To apply current diagnostic criteria for PFAPA syndrome in a group of 359 adults with unexplained recurrent fevers monitored in our Unit between January 2007 and June 2011.
RESULTS:
We have found 17 out of 359 patients fulfilling the diagnosis of PFAPA syndrome: these patients (10 males, 7 females) were Caucasian with a mean age of 33.3±9.5 years, had recurrent febrile episodes begun at a mean age of 25.9±8.3 years and a mean number of episodes of 8.3±5.2 per year with a mean duration of 5.5±1.8 days. In particular, 7/17 patients had the 3 cardinal signs, the other 10 had a combination of 2 signs. Corticosteroids were given in 14/17 patients; tonsillectomy was performed in 9/17 patients: corticosteroid responsiveness and tonsillectomy efficacy were observed respectively in 11 and 2 patients.
CONCLUSIONS:
Our case highlights the importance of considering PFAPA syndrome in adults presenting with unexplained recurrent fevers and symptoms commonly encountered in general medical practice.