Characteristics and Disease Course in a Cohort of Children With PFAPA Syndrome in the Community of Madrid, Spain

Alcalde, María de las Mercedes Ibáñez; Asenjo, Laura Caldevilla; Rey, Cristina Calvo; Marañés, Fernando García-Mon; Gamero, Daniel Blázquez; Lozano, J. Saavedra; Gómez, María Luisa Navarro; Matos, T. Hernández-Sampelayo; Sebastián, Mar Santos

doi:10.1016/j.reumae.2017.10.004

Cited by 6 publications

(11 citation statements)

References 26 publications

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“…27,33,34,37,39 In our cohort, the younger the age at diagnosis, the shorter the asymptomatic interval between flares (27 days vs 38 days), another finding that has been reported by other studies. 39,40 On the other hand, a comparison between the clinical presentation of paediatric and adult patients with PFAPA has found that the frequency of the flares was significantly higher in paediatric cases, while febrile attack duration was significantly longer in adults. 22,41 The frequency of characteristic symptoms in our group showed higher incidence of cervical adenitis and aphthous stomatitis.…”

Section: Clinical Findingsmentioning

confidence: 99%

“…Elevation of inflammatory parameters and normal values of procalcitonin during flares was present in our group, and these findings are consistent with previously published data. 39,40,[45][46][47][48] Many studies have found that the level of procalcitonin during episodes of PFAPA is lower compared to bacterial infections, and this finding may be useful in distinguishing febrile episodes of PFAPA from bacterial infections in children. [47][48][49][50] Complete blood cell counts during febrile episodes in our cohort revealed a raising of leucocyte, neutrophil and monocyte levels, as well as the reduction of thrombocytes.…”

Section: Laboratory Findingsmentioning

confidence: 99%

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PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

Lazea¹,

Damian²,

Vulturar³

et al. 2022

IJGM

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The aim of this study is to describe a group of Romanian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Materials: This consisted of 39 children diagnosed with PFAPA syndrome according to Thomas' criteria (eight patients with an age at diagnosis <1 year and 31 patients with an age at diagnosis >1 year). Methods: Retrospective analysis of the patients with PFAPA syndrome was focused on clinical features, laboratory findings and therapeutic methods. Comparison between the two groups divided by age at onset was also investigated. Results: Median age at onset was 1.58 years, and median age at diagnosis was 2.97 years. The mean interval between episodes was 35.5 days and the mean duration per febrile episode was 4.1 days. The median diagnosis delay was 2.42 years. The patients presented pharyngitis (100%), adenitis (94.8%) and aphthous lesions (66.7%). The frequency of febrile attacks was higher in children with an age at diagnosis under 1 year (p = 0.0287). Younger age was associated with the presence of aphthae. The mean value of C-reactive protein (CRP) was 7.9mg/dl and the mean value of leucocytes was 14,839/mm 3 . In 95% of patients given oral corticosteroids, remission of symptoms was reported within 24 h. In three patients, tonsillectomy was performed with complete remission of the disease. Conclusion:We present a cohort of children with PFAPA syndrome, with clinical and laboratory features similar to those described in the literature. Febrile attacks had a higher incidence in children with younger age at the onset of the disease. The patients had a favorable response to corticosteroids.

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Section: Clinical Findingsmentioning

confidence: 99%

Section: Laboratory Findingsmentioning

confidence: 99%

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

Lazea¹,

Damian²,

Vulturar³

et al. 2022

IJGM

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“…They are typically given as a single dose of prednisone at 1–2 mg/kg at the onset of the fever episode and have been shown to be effective in about 80–95% of patients. 4 , 9 , 53 , 64 A lower dose of prednisone at 0.5 mg/kg has been demonstrated to be effective, 65 as well as betamethasone at 0.1–0.2 mg/kg. 43 Amarilyo et al found that the majority of physicians use 1 mg/kg of prednisone to treat attacks, and recommend increasing to 2 mg/kg in the case of frequent fever flares or incomplete response to the lower dose.…”

Section: Treatment Optionsmentioning

confidence: 99%

“…It may also have the untoward effect of causing the fever cycles to occur more frequently over time in some patients. 4 , 9 , 29 , 53 , 64 , 67 It is not well-known what the effect is of repeated monthly doses over multiple years. Given the potential side effects of long-term use of steroids, we recommended usage for short-term symptomatic relief and as a diagnostic tool, in particular to rule out an infectious cause.…”

Section: Treatment Optionsmentioning

confidence: 99%

“…15 There is some evidence supporting the efficacy of surgical over medical treatment 73,74 ; however, other cohorts of patients have found a similar time to remission in the two approaches. 64,75 Additionally, some patients may not respond fully to tonsillectomy, which in some cases has been linked to mutations in the MEFV gene. Pehlivan et al 62 found a significant difference in response to surgical treatment between patients with PFAPA who were heterozygous for MEFV mutations and those who were not: among patients who did not respond or responded only incompletely to tonsillectomy, around 50% carried variants in MEFV.…”

Section: Surgical Treatmentmentioning

confidence: 99%

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Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review

Wang

Manthiram

Dedeoğlu

et al. 2021

World j. otorhinolaryngol.-head neck surg.

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Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever condition in children, with most cases appearing by the age of 5. Although PFAPA is generally a self-limited condition, it can have a major impact on a child's quality of life, as well as that of their family. Recent research has continued to shed light on the genetic and immunologic factors that play a role in the pathogenesis of PFAPA. There also exists significant heterogeneity in treatment strategies, and progress has been made to develop evidence-based management strategies and establish a standard of care. This review will outline current knowledge regarding the pathogenesis of PFAPA, as well as treatment strategies and our clinical experience.

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Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

Burton

Pollard

Ramsden

et al. 2019

Cochrane Database of Systematic Reviews

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Background Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a rare clinical syndrome of unknown cause usually identified in children. Tonsillectomy is considered a potential treatment option for this syndrome. This is an update of a Cochrane Review first published in 2010 and previously updated in 2014. Objectives To assess the e ectiveness and safety of tonsillectomy (with or without adenoidectomy) compared with non-surgical treatment in the management of children with PFAPA.

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Characteristics and Disease Course in a Cohort of Children With PFAPA Syndrome in the Community of Madrid, Spain

Cited by 6 publications

References 26 publications

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA)

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