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’s syndrome, PFAPA 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.