Long-term follow-up of tonsillectomy efficacy in children with PFAPA syndrome

Aktaş, Öztürk; Gürbüz, Hande; Calışkan, Sebla; Erdur, Ömer; Cırık, Ahmet Adnan

doi:10.1016/j.bjorl.2017.10.012

Cited by 14 publications

(9 citation statements)

References 28 publications

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“…The long‐term prognosis of PFAPA after tonsillectomy is good. A prospective Turkish series of 23 PFAPA patients reported sustained remission after tonsillectomy in 91% of cases in a one‐year follow‐up . Licameli et al.…”

Section: Resultsmentioning

confidence: 93%

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Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

2019

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Aim: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of a periodic fever in childhood. The exact pathogenesis and the aetiology of PFAPA are still unknown. Methods:We conducted a non-systematic review of published articles about PFAPA syndrome and summarised the evidence for diagnostic criteria and treatment options for PFAPA.Results: The first proposed diagnostic criteria for PFAPA, in addition to periodic fever, included aphthous stomatitis, pharyngitis or cervical lymphadenitis in children younger than five years at the beginning of the symptoms. C-reactive protein (CRP) levels and leucocyte counts increase in most patients during episodes. Recent research reveals that tonsillectomy provides an immediate and long-lasting cure for PFAPA, even in the absence of classic criteria of aphthous stomatitis, pharyngitis or cervical adenitis and in children older than five years. Conclusion:We suggest that PFAPA can be diagnosed in children with at least five regularly occurring fever episodes without any other explanation, even in the absence of aphthous stomatitis, pharyngitis or cervical lymphadenitis and also in children older than five years. Key notesAfter the introduction of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) in 1987, cumulating research evidence has clarified clinical features of the syndrome. Tonsillectomy provides an immediate and long-lasting cure for PFAPA, even in the absence of classic criteria of stomatitis, pharyngitis or cervical adenitis and in children older than five years. It is time to reconsider the diagnostic criteria for PFAPA in future research and in clinical practice.

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Section: Resultsmentioning

confidence: 93%

“…The most distinctive feature of PFAPA is a clockwork periodicity of fevers . In most cases, the episodes can be forecasted within a margin of seven days .…”

Section: Resultsmentioning

confidence: 99%

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

2019

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“…In a recent study of 23 patients, 91% of patients had complete resolution of PFAPA symptoms immediately after surgery, with 100% of patients achieving resolution within 3 months [48]. Finally, a Cochrane review of randomized controlled trials (n = 65) comparing surgery versus corticosteroids or expectant management found complete resolution in all tonsillectomy patients by the end of 6 months [37]. In this study of 87 patients, there was a 73.1% success rate of tonsillectomies resolving episodes, which correlates well with previous literature results.…”

Section: Resultsmentioning

confidence: 98%

“…Tonsillectomy has been shown to be helpful. In a recent Cochrane review, there was a relative risk reduction of 4.38 in PFAPA symptoms and an overall reduction in both severity and frequency of episodes post-tonsillectomy [37]. Demonstrating a possible link between FMF and PFAPA, colchicine, an anti-inflammatory medication commonly used in the treatment of FMF, has been shown to increase the interval between episodes but does not reduce the severity and has been reserved for patients whose symptoms do not resolve following tonsillectomy [35].…”

Section: Research Articlementioning

confidence: 99%

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

J¹,

Jonathan²,

Lee³

et al. 2018

Int J Aller Medications

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Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is characterized by periodic cyclical fever, cervical adenopathy, pharyngitis, and aphthous stomatitis. Administration of oral corticosteroids at the onset of fever is the current initial treatment. Tonsillectomy resolves PFAPA symptoms in 50-100% of patients. Objective: The goal of this retrospective observational study was to characterize aspects of PFAPA patients seen at a medium size children's hospital between 2008 and 2016 and evaluate treatment outcomes.

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“…Moreover, after their use interval between flares can become shorter. Tonsillectomy also demonstrated a good result in the majority of cases [16]. If flare episodes are frequent and / or tonsillectomy is ineffective, colchicine may be used to increase the interval between episodes [17].…”

Section: Introductionmentioning

confidence: 95%

Clinical features and diagnosis of PFAPA syndrome: approach of the primary care physician

Burbela¹,

Volianska²,

Boyarchuk³

2021

polp

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Introduction: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA), described for the first time in 1987 by Marshall, is an inflammatory disease that is characterized by febrile episodes with a specific symptoms. PFAPA syndrome is the most frequent among autoinflammatory diseases. The purpose of this study was to evaluate the clinical features of PFAPA syndrome in children in Western Ukraine and its timely diagnosis by the primary care physicians. Material and methods:The medical records of 28 children with PFAPA syndrome referred to the inpatient and outpatient departments of Ternopil Regional Children's hospital (tertiary pediatric care hospital) between 2015 and 2020 were reviewed. Results: The mean age of disease onset was 2.9 ±1.5 years, ranged from 1 to 6 years.The disease occurred more often in male (71.4%, p = 0.0013). Besides fever, the majority of patients were presented with pharyngitis (100%), adenitis (64.3%) and aphtous stomatitis (53.6%). The effectiveness of steroid drugs occurred in all patients: in 25 (89.3%) -full response, in 3 (10.7%) -partial response. Tonsillectomy was performed in 2 cases (7.1%) and only in one of them PFAPA symptoms have passed. PFAPA syndrome was diagnosed 1.2 ±0.5 years from the onset of the first symptoms, mainly by the specialists of tertiary pediatric care. Conclusions:The combination of recurrent fever with pharyngitis, adenitis, and aphthous stomatitis may be a clue to the suspicion of PFAPA by primary care physicians who need to seek professional help to rule out other (genetic, metabolic, self-inflammatory, etc.) causes, as this diagnosis is an exclusion diagnosis. There is a need to raise awareness among primary care physicians about PFAPA, which can improve timely diagnosis and treatment, and avoid unnecessary testing and treatment.

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Long-term follow-up of tonsillectomy efficacy in children with PFAPA syndrome

Abstract: Tonsillectomy is a good option for the treatment of PFAPA syndrome.

Cited by 14 publications

References 28 publications

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

Clinical features and diagnosis of PFAPA syndrome: approach of the primary care physician

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