Periodic fever, aphthous stomatitis, pharyngitis and adenitis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:
Synonyms and keywords: Marshall Syndrome
Overview
Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is a medical condition, typically starting in young children, in which high fever occurs periodically at intervals of about 3-5 weeks, frequently accompanied by aphthous ulcers, pharyngitis and/or adenitis. The syndrome was described in 1987 and named two years later.[1][2][3]
Historical Perspective
Classification
- There is no established system for the classification of periodic fever, aphthous stomatitis, pharyngitis and adenitis.
Pathophysiology
- The exact pathogenesis of periodic fever, aphthous stomatitis, pharyngitis and adenitis is not fully understood.
Causes
- PFAPA is frequently discussed together with other periodic fever syndromes, but it is unknown whether the cause is primarily genetic or due to an initial infection.[3]
Differentiating Periodic fever, aphthous stomatitis, pharyngitis and adenitis from Other Diseases
- Periodic fever, aphthous stomatitis, pharyngitis and adenitis must be differentiated from Behcet disease, differential dx2, and [differential dx3].
Epidemiology and Demographics
- The incidence of PFAPA syndrome is approximately [number range] per 100,000 individuals worldwide.
- The prevalence of PFAPA syndrome is approximately [number range] per 100,000 individuals worldwide.
- There is no racial predilection to PFAPA syndrome.
Risk Factors
- There are no established risk factors for PFAPA syndrome.
Screening
- There is insufficient evidence to recommend routine screening for PFAPA syndrome.
Natural History, Complications, and Prognosis
- According to present research, PFAPA does not lead to other diseases and spontaneously resolves as the child gets older, with no long term effects.[2]
Diagnosis
Diagnostic Study of Choice
- There are no established criteria for the diagnosis of PFAPA syndrome.
History and Symptoms
Signs and symptoms
The key symptoms of PFAPA are those in its name: periodic high fever at intervals of about 3-5 weeks, as well as aphthous ulcers, pharyngitis and/or adenitis. In between episodes, and even during the episodes, the children appear healthy.[3]
Physical Examination
Laboratory Findings
Electrocardiogram
- There are no ECG findings associated with PFAPA syndrome.
X-ray
- There are no x-ray findings associated with PFAPA syndrome.
Echocardiography or Ultrasound
- There are no echocardiography/ultrasound findings associated with PFAPA sayndrome.
CT Scan
- There are no CT scan findings associated with PFAPA syndrome.
MRI
- There are no MRI findings associated with PFAPA syndrome.
Other Imaging Findings
- There are no other imaging findings associated with PFAPA syndrome.
Other Diagnostic Studies
- There are no other diagnostic studies associated with PFAPA syndrome.
Treatment
Medical Therapy
- There is no treatment for PFAPA syndrome; the mainstay of therapy is supportive care.
- A possible treatment for PFAPA is a single dose of prednisone (2 mg per kg body mass) at the beginning of each fever episode.
- The single-dose usually terminates the fever within several hours. However, in some children, prednisone causes fever episodes to occur more frequently (and more regularly).[2]
Surgery
- Surgical intervention is not recommended for the management of PFAPA syndrome.
Primary Prevention
There are no established measures for the primary prevention of PFAPA syndrome.
Secondary Prevention
There are no established measures for the secondary prevention of PFAPA syndrome.
References
- ↑ Thomas, Kenneth Tyson (1999). "Periodic fever syndrome in children". Journal of Pediatrics. Mosby, Inc. 135: 1–5. Retrieved 2008-03-07. Unknown parameter
|coauthors=ignored (help) - ↑ 2.0 2.1 2.2 Padeh, Shai (1999). "Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: Clinical characteristics and outcome". Journal of Pediatrics. Mosby, Inc. 135: 98–101. Retrieved 2008-03-07. Unknown parameter
|coauthors=ignored (help) - ↑ 3.0 3.1 3.2 Long, Sarah S. (1999). "Syndrome of Periodic Fever, Aphthous stomatitis, Pharyngitis, and Adenitis (PFAPA) - What it isn't. What is it?". Journal of Pediatrics. Mosby, Inc. 135: 1–5. Retrieved 2008-03-07.