PFAPA syndrome: new clinical aspects disclosed

Tasher, Diana; Somekh, Eli; Dalal, Ilan

doi:10.1136/adc.2005.084731

Cited by 165 publications

(150 citation statements)

References 18 publications

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“…Tonsillectomy is generally recommended for patients aged three years and older to avoid postoperative complications, in accordance with the clinical guideline 13 . Febrile attacks of PFAPA syndrome can begin as early as 1 month of age 1,3,11,14 . Younger patients with PFAPA syndrome should wait until 3 years of age for tonsillectomy.…”

Section: Discussionmentioning

confidence: 99%

Tonsillectomy as Treatment for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

Taniguchi¹,

Sakai²,

Ichiyama³

et al. 2017

J. Nepal Paedtr. Soc.

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Although the etiology of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome remain unclear, various treatments have been reported. We here describe two thought-provoking cases. Our experience suggests that tonsillectomy is effective against PFAPA syndrome, and can safely be performed in patients three years and older. We recommend that tonsillectomy be considered as early as possible in patients with PFAPA syndrome.

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Section: Discussionmentioning

confidence: 99%

Tonsillectomy as Treatment for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

Taniguchi¹,

Sakai²,

Ichiyama³

et al. 2017

J. Nepal Paedtr. Soc.

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“…This rapid response has a diagnostic feature that also helps to distinguish PFAPA syndrome from other periodic fever syndromes (6,20). However, glucocorticoids have been shown to shorten the time between attacks in 25% of patients (26). Symptoms may recur within 48-72 hours after the first dose (27).…”

Section: Treatmentmentioning

confidence: 99%

Current Diagnosis and Treatment Models of Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Lymphadenitis Syndrome

Kısaarslan¹,

Ünsür²,

Sözeri³

2017

jpr

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“…As the etiology ofPFAPA is still unknown, therapy is empiric and it is important to note that PFAPAshould not be misdiagnosed as recurrent upper respiratory tract infections. Case series have been described, and oral corticosteroids seem to be the most effective nonsurgical treatment (37,38).…”

Section: Periodic Fever Aphthous Stomatitis Pharingytis and Cervicamentioning

confidence: 99%

“…PFAPA is a syndrome characterised by stomatitis with a cluster ofaphthous lesions, pharyngitis, cervical adenitis and high fever occurring every 30 days (range 15-50) and lasting for four days (range 2-7) (37,38). As the etiology ofPFAPA is still unknown, therapy is empiric and it is important to note that PFAPAshould not be misdiagnosed as recurrent upper respiratory tract infections.…”

Section: Periodic Fever Aphthous Stomatitis Pharingytis and Cervicamentioning

confidence: 99%

Use of Topical or Systemic Steroids in Children with Upper Respiratory Tract Infection

et al. 2013

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Steroids have long been used to improve a number of clinical conditions because of their role in reducing inflammatory responses, but their use has always been limited because of their possible longterm side effects. The aim of this review is to establish whether steroids can have a positive effect on the outcome of some pediatric upper respiratory tract infections. We used PubMed to select all ofthe studies on topical or systemic steroids, and their therapeutic use in children with rhinosinusitis (RS), acute otitis media (AOM), otitis media with effusion (OME), acute pharyngitis (AP), or periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA), published over the last 15 years. Although a generally significant improvement in signs and symptoms has been observed with the use of intranasal steroids in children with RS, it is not clear which molecule should be recommended, how long treatment should be continued, or whether the benefits are limited to allergic children. No high-quality studies on the use of topical or systemic steroids in AOM have been carried out, and the results of studies of OME are controversial. The potential positive effects of steroids in children with AP are too slight to justify their use, and their possible adverse effects (particularly in the case of repeated administration) have not been clearly evaluated. Oral corticosteroids seem to be effective in resolving the symptoms of PFAPA, although they do not prevent future fever cycles. These findings show that further randomised and controlled studies are required in order to approach upper respiratory tract infections correctly and avoid the risks associated with frequent steroid use.Steroids have long been used to improve a number ofclinical conditions because oftheir role in reducing inflammatory responses (l). Inflammation is a natural host response to an infection, but an excessive response can worsen the clinical condition. Steroids act by switching off multiple inflammatory genes, such as those regulating the secretion of cytokines, chemokines, adhesion molecules and inflammatory enzymes, activating a number of anti-inflammatory genes, and increasing the degradation of the mRNA encoding certain inflammatory proteins (2).However, the use of systemic steroids has

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PFAPA syndrome: new clinical aspects disclosed

Abstract: We describe several new characteristics of PFAPA syndrome in children, contributing to our knowledge of this relatively unrecognised but troublesome syndrome. Early diagnosis and appropriate treatment can markedly improve the quality of life of both patients and families.

Cited by 165 publications

References 18 publications

Tonsillectomy as Treatment for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

Tonsillectomy as Treatment for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

Current Diagnosis and Treatment Models of Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Lymphadenitis Syndrome

Use of Topical or Systemic Steroids in Children with Upper Respiratory Tract Infection

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