Periodic fever, aphthous stomatitis, pharyngitis and adenitis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sahar Memar Montazerin, M.D.[2]
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.
Historical Perspective
- Periodic fever, aphthous stomatitis, pharyngitis and adenitis was first discribed by Dr. Marshall in 1987.[1]
- Initially it was named fever, aphthous stomatitis, pharyngitis and adenitis (FAPA) syndrome, later was changed to periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to further emphasize on its periodic nature, the main feature of this disorder.[2]
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 cyclic neutropenia, familial Mediterranean fever (FMF), familial Hibernian fever (FHF), and, hyperglobulinemia D syndrome.[4][5][6][7][8][9]
Epidemiology and Demographics
- The incidence of PFAPA syndrome is approximately 23 per 100,000 children up to 5 years of age worldwide.[10]
- The exact prevalence of PFAPA syndrome is not known, however it appears to be more frequent than other autoinflammatory disorders.[11][12]
- There is no racial predilection to PFAPA syndrome.
- PFAPA syndrome affects men and women equally.
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.[13]
Diagnosis
Diagnostic Study of Choice
- The diagnosis of PFAPA syndrome is made when the following diagnostic criteria are met:[6]
| Diagnostic criteria of PFAPA |
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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).[13]
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
- ↑ Marshall, Gary S.; Edwards, Kathryn M.; Butler, Joseph; Lawton, Alexander R. (1987). "Syndrome of periodic fever, pharyngitis, and aphthous stomatitis". The Journal of Pediatrics. 110 (1): 43–46. doi:10.1016/S0022-3476(87)80285-8. ISSN 0022-3476.
- ↑ "PFAPA SYNDROME : The Pediatric Infectious Disease Journal".
- ↑ 3.0 3.1 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.
- ↑ Feder, Henry M. (1992). "Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis". The Pediatric Infectious Disease Journal. 11 (4): 318–321. doi:10.1097/00006454-199204000-00011. ISSN 0891-3668.
- ↑ Dahn KA, Glode MP, Chan KH (September 2000). "Periodic fever and pharyngitis in young children: a new disease for the otolaryngologist?". Arch. Otolaryngol. Head Neck Surg. 126 (9): 1146–9. PMID 10979131.
- ↑ 6.0 6.1 Thomas, Kenneth Tyson; Feder, Henry M.; Lawton, Alexander R.; Edwards, Kathryn M. (1999). "Periodic fever syndrome in children". The Journal of Pediatrics. 135 (1): 15–21. doi:10.1016/S0022-3476(99)70321-5. ISSN 0022-3476.
- ↑ Scimeca PG, James-Herry AG, Weinblatt ME (May 1996). "Atypical PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) in a young girl with Fanconi anemia". J. Pediatr. Hematol. Oncol. 18 (2): 159–61. PMID 8846129.
- ↑ Padeh, Shai; Brezniak, Naphtali; Zemer, Debora; Pras, Elon; Livneh, Avi; Langevitz, Pnina; Migdal, Amyel; Pras, Mordechai; Passwell, Justen H. (1999). "Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: Clinical characteristics and outcome". The Journal of Pediatrics. 135 (1): 98–101. doi:10.1016/S0022-3476(99)70335-5. ISSN 0022-3476.
- ↑ Sohar, Ezra; Gafni, Joseph; Pras, Mordehai; Heller, Harry (1967). "Familial Mediterranean fever". The American Journal of Medicine. 43 (2): 227–253. doi:10.1016/0002-9343(67)90167-2. ISSN 0002-9343.
- ↑ Førsvoll, Jostein; Kristoffersen, Einar Klaeboe; Øymar, Knut (2013). "Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study". Acta Paediatrica. 102 (2): 187–192. doi:10.1111/apa.12069. ISSN 0803-5253.
- ↑ Hofer, Michaël; Mahlaoui, Nizar; Prieur, Anne-Marie (2006). "A child with a systemic febrile illness – differential diagnosis and management". Best Practice & Research Clinical Rheumatology. 20 (4): 627–640. doi:10.1016/j.berh.2006.04.001. ISSN 1521-6942.
- ↑ Semianchuk VB (2017). "Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (pfapa) syndrome in children". Wiad. Lek. 70 (1): 144–147. PMID 28343210.
- ↑ 13.0 13.1 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
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