Differential cytokine profiles in pediatric patients with PFAPA syndrome and recurrent tonsillitis

Nakano, Seiichi; Kondo, Eiji; Iwasaki, Hidetaka; Akizuki, Hironori; Matsuda, Kazunori; Azuma, Takehiko; Sato, Go; Kitamura, Yoshiaki; Abe, Koji; Takeda, Noriaki

doi:10.2152/jmi.68.38

Cited by 3 publications

(1 citation statement)

References 19 publications

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“…[ 20 ] Moreover, compared to those with recurrent tonsillitis, PFAPA children exhibited elevated TH1 and reduced T helper 2 (TH2) cytokine levels during flare attacks, while the former group of patients were associated with elevated TH2 cytokine secretion only. [ 21 ] Similar oscillation in cytokine and chemokine levels, with increased production of proinflammatory mediators, was observed during afebrile phases,[ 22 ] which were also reported to display elevated tonsillar cluster of differentiation (CD)8+ T cell levels. [ 23 ] In contrast to the studies on the cellular branch of adaptive immunity, literature on antibody production in PFAPA subjects is still not consistent.…”

Section: Introductionmentioning

confidence: 88%

Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

Gazi,

Dalkan,

Sanlidag

et al. 2023

Arch Rheumatol

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Objectives: This study aimed to extend the literature by analyzing immunoglobulin (Ig) A, IgE, IgG, IgG2, IgG3, and IgM antibody levels in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) patients. Patients and methods: This study retrospectively analyzed the antibody test results of 20 pediatric patients (10 males, 10 females; mean age: 2.5±1.5 years; range, 0.5 to 5.4 years) with and without flare who were initially evaluated for a number of underlying diseases due to periodic fever/infectious symptoms but then diagnosed with PFAPA between January 2015 and December 2020. Antibody levels were determined by chemiluminescence microparticle immunoassay. The results were retrospectively compared with a group of healthy children after the PFAPA diagnosis was confirmed. Results: The chemiluminescence microparticle immunoassay revealed 35%, 65%, 20%, 86.6%, and 55% of PFAPA cases with low serum levels of IgA, IgG, IgG2, IgG3, and IgM respectively, while 56.2% had high IgE levels. Moreover, low serum levels of at least two antibody classes or subclasses were reported in 80% of the PFAPA children. While cases with low IgG serum levels were with the highest incidence rates among the low IgG3 PFAPA patient population, both high IgE and low IgM cases were common in the rest of the patients. Conclusion: Our results suggest an association between PFAPA and low serum antibody levels, particularly of IgG3. Future studies are needed to confirm our conclusion.

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Section: Introductionmentioning

confidence: 88%

Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

Gazi,

Dalkan,

Sanlidag

et al. 2023

Arch Rheumatol

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The role of inflammatory mediators in the pathogenesis of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome

et al. 2022

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Adult‐Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome on the Basis of Selective IgA Deficiency

Altiner,

Ekinci

2024

Case Reports in Dermatological Medicine

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Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is an autoinflammatory disease that is thought to occur with the contribution of genetic and environmental factors, but its etiology has not been clearly elucidated. It is characterized by recurrent attacks with fever, pharyngitis, oral aphthous lesions, and cervical lymphadenopathy, and an increase in the level of serum acute phase reactants is observed during the attacks. Although PFAPA usually begins in childhood, adult‐onset cases are also reported in the literature. In the pathogenesis of PFAPA, an increase in the expression of various inflammatory cytokines, especially interleukin‐1β (IL‐1β), is observed as a result of the increase in inflammasome activity. Selective IgA deficiency (SIgAD) is the most prevalent primary immunodeficiency. Although most SIgAD cases remain asymptomatic and remain undiagnosed, it is known that the risk of mucosal infection is generally increased in SIgAD cases. In addition, the frequency of autoinflammatory diseases is increased in SIgAD cases compared with the general population. We aim to present a case of adult‐onset PFAPA and SIgAD coexistence.

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Differential cytokine profiles in pediatric patients with PFAPA syndrome and recurrent tonsillitis

Cited by 3 publications

References 19 publications

Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis

The role of inflammatory mediators in the pathogenesis of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome

Adult‐Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome on the Basis of Selective IgA Deficiency

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