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


OBJECTIVE
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.
Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Unit of Rheumatology, Policlinico Le Scotte, University of Siena, Italy. cantariniluca@hotmail.com
Luca
L
Cantarini
Antonio
A
Vitale
Beatrice
B
Bartolomei
Mauro
M
Galeazzi
Donato
D
Rigante
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005260:Female; D005334:Fever / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007558:Italy; D008199:Lymphadenitis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D010612:Pharyngitis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D012189:Retrospective Studies; D013281:Stomatitis, Aphthous / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D013577:Syndrome; D013997:Time Factors; D014068:Tonsillectomy; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones