PFAPA syndrome (fever, aphthous stomatitis, pharyngitis and cervical lymphadenitis [1] , or Marshall syndrome is a chronic disease of unknown etiology, manifested mainly in children. The main characteristic of the syndrome is episodes of increased body temperature, occurring from 3 to 5 weeks These episodes are often accompanied by aphthous stomatitis , pharyngitis and cervical lymphadenitis . This syndrome was first described in 1987. It received its present name two years later [2] [3] [4] .
| Syndrome of PFAPA (fever, aphthous stomatitis, pharyngitis and cervical lymphadenitis | |
|---|---|
| ICD-10 | |
| ICD-10-KM | |
| DiseasesDB | 33682 |
Content
Reasons to
The exact causes of the disease are unknown. [5] The ability of a single dose of corticosteroids to stop an attack of PFAPA indicates an involvement in the development of the disease proinflammatory cytokines , rather than infection. [6]
Diagnosis
PFAPA syndrome has the following diagnostic features: [6] [7]
- Return febrile fever (38-41 °) in the absence of upper respiratory tract infection
- Exudative tonsillitis in combination with negative smear on microflora
- Cervical Lymphadenitis
- Pharyngitis
- Often aphthous stomatitis
- Complete absence of symptoms between bouts of the disease
- Children have normal growth and development.
- Fast (within 2-4 hours) response to a single dose of corticosteroids
Treatment
The attacks of the disease usually go away on their own in 4-10 days. Drug treatment is prescribed to reduce the severity of attacks. [8] A single dose of corticosteroids at the very beginning of the disease usually stops the attack within a few hours, but in some children it may be the cause of more frequent episodes of the disease. [five]
Tonsillectomy is used as an alternative to medical treatment, but the results are satisfactory ambiguous. [8] [6]
Forecast
According to the available data, PFAPA syndrome does not lead to additional diseases or complications and disappears without a trace when children get older [3] . However, in adults, the disease may not disappear on its own or reappear many years later. [6]
Notes
- ↑ Syndrome PFAPA (fever, aphthous stomatitis, pharyngitis and cervical lymphadenitis) / Medicus. Embassy of Medicine
- ↑ Thomas, Kenneth Tyson; Feder, Lawton, Edwards. Periodic fever syndrome in children (Neopr.) // Journal of Pediatrics . - Mosby, Inc., 1999. - T. 135 . - p . 1-5 . - DOI : 10.1016 / S0022-3476 (99) 70316-1 .
- ↑ 1 2 Padeh, Shai; (eight others). Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: Clinical characteristics and outcome (Eng.) // Journal of Pediatrics : journal. - Mosby, Inc., 1999. - Vol. 135 , no. 1 . - P. 98-101 . - DOI : 10.1016 / S0022-3476 (99) 70335-5 . - PMID 10393612 . Archived February 22, 2013.
- ↑ Long, Sarah S. Syndrome of Periodic Fever, Aphthous stomatitis, Pharyngitis, and Adenitis (PFAPA) - What it isn't. What is it? (English) // Journal of Pediatrics : journal. - Mosby, Inc., 1999. - Vol. 135 . - P. 1-5 . - DOI : 10.1016 / S0022-3476 (99) 70316-1 . Archived May 12, 2008.
- ↑ 1 2 Vigo, G; Zulian, F. Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA). (Eng.) // Autoimmunity reviews: journal. - 2012. - November ( vol. 12 , no. 1 ). - P. 52—5 . - DOI : 10.1016 / j.autrev.2012.07.021 . - PMID 22878272 .
- ↑ 1 2 3 4 Padeh, S; Stoffman, N; Berkun, Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults. (Eng.) // The Israel Medical Association journal: IMAJ: journal. - 2008. - May ( vol. 10 , no. 5 ). - P. 358-360 . - PMID 18605359 .
- ↑ Kuzmina, N.N .; Movsisyan, G.R. PFAPA (Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis - Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) or Marshall syndrome in children. (rus.) // Scientific-practical rheumatology: journal. - 2005. - № 5 . - S. 80-83 . - DOI : 10.14412 / 1995-4484-2005-50 .
- 2 1 2 Burton MJ, Pollard AJ, Ramsden JD, Chong LY, Venekamp RP Tonsillectomy for a periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) (English) // Cochrane Database Syst Rev: journal: journal. - 2014. - No. 9 - P. CD008669 . - DOI : 10.1002 / 14651858.CD008669.pub2 . - PMID 25209127 .
Literature
- Marshall GS, Edwards KM, Butler J., Lauton AR Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J. Pediatr. 1987, 110 (1): p 43−46.