Medicine:Periodic fever, aphthous stomatitis, pharyngitis and adenitis

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Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis
Other namesPeriodic fever aphthous pharyngitis and cervical adenopathy (PFAPA)
SpecialtyPediatric, Rheumatology, Immunology
SymptomsFever recurring on a ~2-6 week cycle
TreatmentTonsillectomy
MedicationCorticosteroids, Colchicine, Cimetidine

Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a medical condition, typically occurring in young children, in which high fever occurs periodically at intervals of about 3-5 weeks, frequently accompanied by aphthous-like ulcers, pharyngitis and/or cervical adenitis (cervical lymphadenopathy). The syndrome was described in 1987 and named two years later.[1] [2][3]

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. At least 6 months of episodes. Diagnosis requires recurrent negative throat cultures and that other causes (such as EBV, CMV, FMF) be excluded.[3]

Cause

The cause of PFAPA is unknown.[4] It is frequently discussed together with other periodic fever syndromes.[3]

Possible causes include primarily genetic factors or it may be due to an initial infection.

The condition appears to be the result of a disturbance of innate immunity.[5] The changes in the immune system are complex and include increased expression of complement related genes (C1QB, C2, SERPING1), interleukin-1-related genes (interleukin-1B, interleukin 1 RN, CASP1, interleukin 18 RAP) and interferon induced (AIM2, IP-10/CXCL10) genes. T cell associated genes (CD3, CD8B) are down regulated. Flares are accompanied by increased serum levels of activated T lymphocyte chemokines (IP-10/CXCL10, MIG/CXCL9), G-CSF and proinflammatory cytokines (interleukin 6, interleukin 18). Flares also manifest with a relative lymphopenia. Activated CD4(+)/CD25(+) T-lymphocyte counts correlated negatively with serum concentrations of IP-10/CXCL10, whereas CD4(+)/HLA-DR(+) T lymphocyte counts correlated positively with serum concentrations of the counterregulatory IL-1 receptor antagonist.[citation needed]

Diagnosis

Treatment

PFAPA syndrome typically resolves spontaneously. Treatment options are used to lessen the severity of episodes.[6] These treatments are either medical or surgical:

One treatment often used is a dose of a corticosteroid at the beginning of each fever episode.[4] A single dose usually ends the fever within several hours.[4] However, in some children, they can cause the fever episodes to occur more frequently.[4] Interleukin-1 inhibition appears to be effective in treating this condition.[5]

There has been some evidence for the use of medications to reduce the frequency of flare-ups, including colchicine and cimetidine.[7]

Surgical removal of the tonsils appears to be beneficial compared to no surgery in symptom resolution and number of future episodes.[6] The evidence to support surgery is; however, of moderate quality.[6]

Children with PFAPA have an impaired quality of life, which may be treated via individual counseling.[8]

Prognosis

According to present research, PFAPA does not lead to other diseases and spontaneously resolves as the child gets older, with no long term physical effects.[2] [9] [10] However, PFAPA has been found in adults and may not spontaneously resolve.[11][non-primary source needed] Children with PFAPA experience lower physical, emotional, and psychosocial functioning.[8] Their performance in school is also substantially impacted.[8]

References

  1. ↑ "Periodic fever syndrome in children". The Journal of Pediatrics 135 (1): 15–21. July 1999. doi:10.1016/S0022-3476(99)70321-5. PMID 10393598. 
  2. ↑ 2.0 2.1 "Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome". The Journal of Pediatrics (Mosby) 135 (1): 98–101. July 1999. doi:10.1016/S0022-3476(99)70335-5. PMID 10393612. http://padeh.net/Jpedpfapa.htm. 
  3. ↑ 3.0 3.1 3.2 "Syndrome of Periodic Fever, Aphthous stomatitis, Pharyngitis, and Adenitis (PFAPA)--what it isn't. What is it?". The Journal of Pediatrics (Mosby) 135 (1): 1–5. July 1999. doi:10.1016/S0022-3476(99)70316-1. PMID 10393593. 
  4. ↑ 4.0 4.1 4.2 4.3 "Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA)". Autoimmunity Reviews 12 (1): 52–55. November 2012. doi:10.1016/j.autrev.2012.07.021. PMID 22878272. 
  5. ↑ 5.0 5.1 "Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade". Proceedings of the National Academy of Sciences of the United States of America 108 (17): 7148–7153. April 2011. doi:10.1073/pnas.1103681108. PMID 21478439. Bibcode: 2011PNAS..108.7148S. 
  6. ↑ 6.0 6.1 6.2 "Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)". The Cochrane Database of Systematic Reviews 2019 (12): CD008669. December 2019. doi:10.1002/14651858.CD008669.pub3. PMID 31886897. 
  7. ↑ "PFAPA syndrome: a review on treatment and outcome". Pediatric Rheumatology Online Journal 14 (1): 38. June 2016. doi:10.1186/s12969-016-0101-9. PMID 27349388. 
  8. ↑ 8.0 8.1 8.2 "Health-related quality of life in children with PFAPA syndrome". Orphanet Journal of Rare Diseases 13 (1): 132. August 2018. doi:10.1186/s13023-018-0878-3. PMID 30092788. 
  9. ↑ "PFAPA Syndrome - Pediatrics". The Merck Manual. September 2022. http://www.merck.com/mmpe/sec19/ch297/ch297g.html. 
  10. ↑ "Periodic Fever Syndrome". Cleveland Clinic. 18 January 2019. http://my.clevelandclinic.org/disorders/periodic_fever_syndrome/rheumatology_overview.aspx. 
  11. ↑ "Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults". The Israel Medical Association Journal 10 (5): 358–360. May 2008. PMID 18605359. http://www.ima.org.il/FilesUpload/IMAJ/0/43/21995.pdf. 

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