Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome:Clinical characteristics and outcome

We report 28 patients (20 male) with a syndrome characterized bq abrupt ons et of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis, and ce rvical adenopathy (PFAPA syndrome). Episodes of fever occurred at intervals of 5.1 +/- 1.3 weeks beginning at the age of 4.2 +/- 2.7 years Fever, mala ise, tonsillitis with negative throat cultures, and cervical adenopathy wer e reported in all 28 patients, aphthae in 19, headache in 5, abdominal pain in 5, and arthralgia in 3. Mild hepatosplenomegaly was observed in 6 patie nts. Mild leukocytosis, elevation of the erythrocyte sedimentation rate, an d fibrinogen were found during attacks. These episodes of illness resolved spontaneously in 4.3 +/- 1.7 days. Serum IgD was found elevated (>100 U/mL) in 12 of the 18 patients tested (140.2 +/- 62.4 U/mL). Affected children g row normally, have no associated diseases, and have no long-term sequelae. Attacks were aborted by a single dose of oral prednisone (2 mg/kg) at the b eginning of the attack in all 15 patients in whom this medication was presc ribed In 9 patients the syndrome has completely resolved (beginning at the age of 2.9 +/- 1.3 and lasting 8 +/- 2.5 years). In 3 other patients comple te resolution of the attacks occurred after tonsillectomy was performed. PF APA is sporadic, and no ethnic predilection was found. Increased awareness of the clinical syndrome has resulted in more frequent diagnosis and adequa te treatment.