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Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy
syndrome: clinical characteristics and outcome.
Padeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, Migdal
A, Pras M, Passwell JH.
Department of Pediatrics, Heller Institute of Medical Research,
Sheba Medical Center, affiliated with the Tel-Aviv University, Sackler
School of Medicine, Tel Hashomer, Israel.
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J Pediatr 1999 Jul;135(1):98-101 Abstract quote
We report 28 patients (20 male) with a syndrome characterized by abrupt
onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis,
and cervical 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, malaise, tonsillitis with negative
throat cultures, and cervical adenopathy were 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 patients. Mild leukocytosis,
elevation of the erythrocyte sedimentation rate, and 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 grow
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 beginning of the attack in all 15 patients in whom this medication
was prescribed. 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
complete resolution of the attacks occurred after tonsillectomy was
performed. PFAPA is sporadic, and no ethnic predilection was found.
Increased awareness of the clinical syndrome has resulted in more frequent
diagnosis and adequate treatment.
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Periodic fever and pharyngitis in young children: a new disease
for the otolaryngologist?
Dahn KA, Glode MP, Chan KH.
Department of Pediatric Otolaryngology, The Children's Hospital,
1056 E 19th Ave, Denver, CO 80218, USA.
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Arch Otolaryngol Head Neck Surg 2000 Sep;126(9):1146-9 Abstract quote
OBJECTIVE: A clinical entity consisting of periodic fever associated
with aphthous stomatitis, pharyngitis and cervical adenitis termed "PFAPA
syndrome" in young children (<5 years old) may be unfamiliar to otolaryngologists.
We present our 5-year experience of PFAPA syndrome.
DESIGN: Case series.
SETTING: Tertiary academic.
PATIENTS: A 5-year retrospective chart review for children (<5 years
old) who have undergone tonsillectomies with and without adenoidectomies
was conducted. Medical records from subjects who underwent the procedures
for recurrent pharyngitis were reviewed with reference to a history
of periodic fever and stomatitis associated with pharyngitis.
INTERVENTIONS: Tonsillectomy with and without adenoidectomy.
MAIN OUTCOME MEASURE: The objective measure was a comparison of the
number of visits to the primary care physician for pharyngitis associated
with fever in a 3-month period before and after the surgical intervention.
The subjective measure was a telephone interview evaluating preoperative
and postoperative symptoms.
RESULTS: Of the 117 patients identified, 22 (19%) underwent surgery
for recurrent pharyngitis. Five subjects (average age, 2.5 years) were
identified as having PFAPA syndrome. The average number of preoperative
PFAPA-related complaints was 11.6 compared with 0.2 for the number of
postoperative PFAPA-related complaints (P=.03).
CONCLUSIONS: Our experience suggests that PFAPA syndrome is an uncommon
disease. Most of these children have undergone workup(s) for sepsis
performed by their pediatricians because of the associated high fever.
The clinical history of this cohort was quite distinctive. This small
sample suggests a significant decrease if not cessation of pharyngitis
following surgical intervention.
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