PFAPA Syndrome in a Population with Endemic Familial Mediterranean Fever

Pehlivan, Esra; Adroviç, Amra; Şahin, Sezgin; Barut, Kenan; Cınar, Ovgu Kul; Kasapçopur, Özgür

doi:10.1016/j.jpeds.2017.08.078

Cited by 56 publications

(32 citation statements)

References 27 publications

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“…The PFAPA Work Group defined patient inclusion characteristics (Table 1) that were slightly modified from the original proposed diagnostic criteria for PFAPA [4][5][6]18]. First, the duration of fever attacks was defined as 3-7 days to avoid confusion with other autoinflammatory syndromes that may share some features with PFAPA but differ in their duration of febrile attacks, such as Familial Mediterranean Fever [19,20]. Second, to exclude recurrent infections and malignancy, patients had to have had more than 6 stereotypical periodic febrile episodes.…”

Section: Resultsmentioning

confidence: 99%

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Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group

Amarilyo

Rothman²,

Manthiram

et al. 2020

Pediatr Rheumatol

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Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA.

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Section: Resultsmentioning

confidence: 99%

“…PFAPA, like other diseases studied by consensus methodology, has no validated response measures, making comparisons between treatment arms challenging [19,20]. Therefore, we established response criteria a priori ( Table 2).…”

Section: Discussionmentioning

confidence: 99%

Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group

Amarilyo

Rothman²,

Manthiram

et al. 2020

Pediatr Rheumatol

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“…Butbul‐Aviel et al reported an association between PFAPA and FMF . FMF should also be considered in patients with PFAPA who do not respond to adenotonsillectomy . Although the association between MEFV gene mutations, such as E148Q or R202Q, and the development of PFAPA remains unclear, attention should be paid to patients with MEFV exon 3 variants with PFAPA symptoms …”

Section: Discussionmentioning

confidence: 99%

Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

et al. 2019

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Aim:The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a common inflammatory disease that presents with periodic fever.We aimed to establish more specific diagnostic criteria for PFAPA based on the clinical characteristics of PFAPA patients in our directory. Method:The clinical, laboratory, genetic, and family history details of 257 Japanese PFAPA patients treated at our and other affiliated hospitals between April 2000 and April 2018 were analyzed along with quantitative measurements of the number of CD64 molecules on neutrophils, and the levels of serum inflammatory cytokines. The sensitivity and specificity of the criteria were calculated for several diseases.Results: Because recurrent fevers were crucial findings, they were defined as the required criterion. Tonsillitis/pharyngitis with white moss were important accompanying signs. Other symptoms associated with febrile episodes were cervical lymphadenitis with tenderness, aphthous stomatitis, sore throat, vomiting, and headache but not cough. A total of 159 (62%) patients had a family history of recurrent fevers, indicating autosomal dominant inheritance. C-reactive protein levels were extremely elevated during febrile attacks but normal in attack-free periods. Serum immunoglobulin D levels were high in 72 of the 199 tested patients. Oral glucocorticoid and cimetidine were extremely effective in all and 51.6% of the patients, respectively. We defined the above as supportive criteria. These criteria were sensitive and specific enough to distinguish PFAPA from other recurrent fever diseases. Raised serum interferon-γ levels and remarkable CD64 expression on neutrophils during flare-ups were recognized, indicating they contributed to diagnosis. Conclusion:Our new criteria are useful for diagnosing PFAPA. K E Y W O R D S cytokine, diagnostic criteria, periodic fever, PFAPA, tonsillitis How to cite this article: Takeuchi Y, Shigemura T, Kobayashi N, et al. Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Int J Rheum Dis.

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“…Other studies have shown a preva-lence of 24.7% to 55.4% of MEFV variants in patients diagnosed with PFAPA, especially in FMF-endemic regions [30][31][32]. Patients with coexistent FMF and PFAPA may have a poorer response to surgical treatment and a better response to colchicine [29,30].…”

Section: Research Articlementioning

confidence: 97%

“…In a study of 359 patients diagnosed with PFAPA by Pehlivan, et al patients with symptoms resembling FMF, who were unresponsive to tonsillectomy, or who had a family history of FMF were investigated further for MEFV gene mutation; 33.8% of these patients were positive for MEFV gene mutation [29]. Other studies have shown a preva-lence of 24.7% to 55.4% of MEFV variants in patients diagnosed with PFAPA, especially in FMF-endemic regions [30][31][32]. Patients with coexistent FMF and PFAPA may have a poorer response to surgical treatment and a better response to colchicine [29,30].…”

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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PFAPA Syndrome in a Population with Endemic Familial Mediterranean Fever

Cited by 56 publications

References 27 publications

Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group

Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group

Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

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