PFAPA is an acronym for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis. The febrile attacks usually last about 4.7 days.
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DISEASE ASSOCIATIONS CHARACTERIZATION
Atypical PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) in a young girl with Fanconi anemia.
Scimeca PG, James-Herry AG, Weinblatt ME.
Division of Pediatric Hematology/Oncology, North Shore University Hospital-Cornell University Medical College, Manhasset, New York 11030, USA.
J Pediatr Hematol Oncol 1996 May;18(2):159-61 Abstract quote
PURPOSE: To describe a case of atypical, severe, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA syndrome) in a patient with Fanconi anemia. Important aspects about the PFAPA syndrome and Fanconi anemia are reviewed.
PATIENTS AND METHODS: An 8-year-old girl with Fanconi anemia was noted to have a pattern of periodic fever, stomatitis, and pharyngitis consistent with the diagnosis of PFAPA syndrome, a generally benign disorder. After prednisone treatment for the syndrome, life-threatening intestinal ulceration and perforation developed, which was successfully treated.
CONCLUSION: In patients with underlying hematologic disease such as Fanconi anemia, PFAPA syndrome may be associated with severe clinical problems in contrast to otherwise normal children with the disorder.
Periodic fever syndrome in children.
Thomas KT, Feder HM Jr, Lawton AR, Edwards KM.
School of Medicine, Vanderbilt University, Nashville, Tennessee, USA.
J Pediatr 1999 Jul;135(1):15-21 Abstract quote
OBJECTIVES: To describe the presentation, clinical course, therapeutic response, and long-term follow-up of patients with a syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA).
STUDY DESIGN: Patients with PFAPA (n = 94) referred over a 10-year period completed a registry form and provided medical records. Follow-up telephone calls were made in late 1997 to determine the persistence of episodes and sequelae.
RESULTS: PFAPA episodes lasted 4.8 days (95% confidence interval 4.5 to 5.1) and recurred every 28 days (confidence interval 26 to 30), with a maximal temperature of 40.5 degrees C (confidence interval 40. 4 degrees to 40.6 degrees ). Of the 83 children available for follow-up, 34 no longer had episodes. In the remainder the episodes did not differ in character but recurred less frequently over time. The affected children had no long-term sequelae. Glucocorticoids were highly effective in controlling symptoms. Tonsillectomy and cimetidine treatment were associated with remission in a small number of patients.
CONCLUSIONS: PFAPA is a not uncommon cause of periodic fever in children. In some children the syndrome resolves, whereas symptoms in others persist. Long-term sequelae do not develop. The syndrome is easily diagnosed when regularly recurring episodes of fever are associated with aphthous stomatitis, pharyngitis, or cervical adenitis.
PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis).
Lee WI, Yang MH, Lee KF, Chen LC, Lin SJ, Yeh KW, Huang JL.
Department of Pediatrics, Chang-Gung Children's Hospital and Chang Gung University, Taoyuan, Taiwan.
Clin Rheumatol 1999;18(3):207-13 Abstract quote
This paper aims to remind paediatric clinicians to suspect and confirm 'PFAPA' syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome).
We report two cases of PFAPA syndrome: a 3-year-old healthy boy with atopic rhinitis and a boy aged 8 years 5 months who simultaneously had lymphocytic vasculitis syndrome treated with immunosuppressive drugs. Both met Marshall's criteria. The literature regarding PFAPA syndrome was complied using a Medline search for articles published between 1963 and 1998 and we then reviewed the reference lists of the articles. The Medline search revealed 28 cases with available clinical manifestations, management and prognosis. Our study describes two additional cases.
We divided the cases into typical (28 cases) and atypical (two cases) PFAPA syndrome. In typical PFAPA, the age of onset was less than 5 years in most cases and the patients presented 4.9 +/- 1.4 days of fever (100%), pharyngitis (89.3%), cervical adenitis (72.1%), stomatitis (71.4%), malaise (64.3%), headache (60.7%), abdominal pain (53.6%) and nausea/vomiting (17.9%). Afebrile intervals were 3.2 +/- 2.4 months and increased with age. The time from initial onset to final episode was 3 years 7 months +/- 3 years 6 months. The total number of episodes was 8.3 +/- 2.5 (range 6-14). Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine. The general outcome was good. In atypical PFAPF, the clinical manifestations were similar to those of typical PFAPA except that the age of onset was more than 5 years, and life-threatening intestinal perforation happened once in a patient with underlying Fanconi's anaemia. It was concluded that typical PFAPA syndrome is benign and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other periodic fever syndromes.
A review of the literatures since the first report in 1987 has shown that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids. One patient with atypical PFAPA, who received low-dose steroids for over 1 year, developed intestinal perforation after an increment of the 7-day steroid dose. If an underlying problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to resolve atypical PFAPA.
Periodic fever, aphthous stomatitis, pharyngitis, adenitis: a clinical review of a new syndrome.
Feder HM Jr.
Department of Family Medicine and Pediatrics, University of Connecticut Health Center, Farmington, USA.
Curr Opin Pediatr 2000 Jun;12(3):253-6 Abstract quote
Periodic fevers (fevers that occur predictably at fixed intervals) are unusual in infants and children.
The classic periodic fever syndrome is cyclic neutropenia (neutropenia followed by infections and fever that recur every 21 days). A new periodic fever syndrome PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) has been characterized over the past decade. PFAPA is defined clinically, because specific laboratory abnormalities have not been found. The clinical characteristic of PFAPA is high fevers (usually 40.0 degrees C to 40.6 degrees C) recurring at fixed intervals every 2 to 8 weeks. The fevers last for about 4 days, then resolve spontaneously. Associated with the fevers are aphthous stomatitis in 70% of patients, pharyngitis in 72% of patients, and cervical adenitis in 88% of patients.
PFAPA is not familial and begins before the age of 5 years. An episode of PFAPA can be aborted with one or two small doses of prednisone. The episodes of PFAPA may last for years and the patient is well between episodes. The cause of PFAPA is unknown and there are no reported sequelae.
PROGNOSIS AND TREATMENT CHARACTERIZATION
Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis.
Feder HM Jr.
Department of Family Medicine and Pediatrics, University of Connecticut Health Center, Farmington 06032.
Pediatr Infect Dis J 1992 Apr;11(4):318-21
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.
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.
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.
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.
J Pediatr 1999;135:98-101.
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