Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults.
BACKGROUND
The new syndrome, known as PFAPA, of periodic fever characterized by abrupt onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenopathy has been described only in pediatric patients. It usually begins before the age of 5 years and in most cases resolves spontaneously before age 10.


OBJECTIVE
To describe a series of adults with PFAPA syndrome.


METHODS
This 6 year retrospective descriptive study includes all newly diagnosed incident adult cases aged 18 years and over referred to our center with symptomatology suggestive of PFAPA syndrome. Patients' medical records were reviewed for past history of the disease, demographic characteristics, symptoms and signs, course of the disease, laboratory findings, and outcome following corticosteroid therapy. The comparison group included our pediatric cohort children (N=320, age 0-18 years) followed for the last 14 years (1994-2008).


RESULTS
Fifteen adult patients were diagnosed with PFAPA syndrome. Episodes of fever occurred at 4.6 +/- 1.3 week intervals, beginning at the age of 20.9 +/- 7.5. All patients had monthly attacks at the peak of the disease, with attacks recurring at 4-8 week intervals over the years. Between episodes the patients were apparently healthy, without any accompanying diseases. Attacks were aborted by a single 60 mg dose of oral prednisone in all patients.


CONCLUSIONS
This study reports the presence of PFAPA syndrome in adult patients. Although the disease is rare, an increased awareness by both patients and family physicians of this clinical syndrome has resulted in more frequent diagnosis in adult patients.
Padeh
Shai
S
Department of Pediatrics A, Safra Children's Hospital, Sheba Medical Center, Tel Hashomer, Israel. shay.pade@sheba.health.gov.il
Stoffman
Nava
N
Berkun
Yackov
Y
D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001799:Blood Sedimentation; D002648:Child; D005260:Female; D005334:Fever / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007964:Leukocytosis / Q000150:complications; D008199:Lymphadenitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D010507:Periodicity; D010612:Pharyngitis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D013281:Stomatitis, Aphthous / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013577:Syndrome
D000893:Anti-Inflammatory Agents; D011241:Prednisone