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Background <p>Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) is a common childhood autoinflammatory disease.</p> Objective <p>This study investigated the influence of early-life factors on attack frequency and treatment resistance in children with PFAPA.</p> Methods <p>Eighty-one children with PFAPA (diagnosed April 2021–2024) were retrospectively evaluated and divided into persistent and non-persistent (self-limiting) groups. Persistent PFAPA was defined as a &lt; 50% reduction in attack frequency since diagnosis, having undergone tonsillectomy for PFAPA, or experiencing ongoing attacks beyond 7 years of age.</p> Results <p>The study included 81 PFAPA patients (female-to-male ratio 0.9; median age 76 months). The patients were divided into two groups: 28 persistent (34.6%) and 53 non-persistent (65.4%). Persistent patients had more frequent attacks (median 6 vs. 1 per year, <i>p</i> &lt; 0.001) of shorter duration (median 3 vs. 4 days, <i>p</i> = 0.003), with less cervical lymphadenopathy (10.7% vs. 30.2%, <i>p</i> = 0.049) and abdominal pain (32.1% vs. 56.6%, <i>p</i> = 0.036). Preterm birth and pacifier use were less common in persistent patients (7.1% vs. 26.4%, <i>p</i> = 0.038; 25% vs. 52.8%, <i>p</i> = 0.016). In multivariate analysis, abdominal pain, preterm birth, and pacifier use independently predicted non-persistence, whereas colchicine use was non-significant.</p> Conclusion <p>Early-life factors, including preterm birth and pacifier use, are associated with a self-limiting PFAPA phenotype.</p> Impact <p><UnorderedList Mark="Bullet"> <ItemContent> <p>This study reveals that specific early-life factors—namely preterm birth and pacifier use—are independently associated with a milder, non-persistent PFAPA course.</p> </ItemContent> <ItemContent> <p>By identifying these perinatal and environmental influences, the research shifts the focus from purely clinical symptoms to the role of early immune maturation in disease trajectory.</p> </ItemContent> <ItemContent> <p>These findings provide clinicians with new tools to predict disease persistence, enabling more personalized management and better-informed parental counseling.</p> </ItemContent> <ItemContent> <p>The association between early-life exposures and PFAPA persistence offers a novel perspective on the pathogenesis of autoinflammatory conditions in children.</p> </ItemContent> </UnorderedList></p>

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Perinatal and early childhood determinants of disease persistence in PFAPA

  • 期刊论文

  • 发表日期：

    2026年5月16日

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  • Elif Benderlioğlu,
  • Gülşah Efeoğlu Gülsoy,
  • Emine Özçelik,
  • Banu Çelikel Acar

摘要

Background

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) is a common childhood autoinflammatory disease.

Objective

This study investigated the influence of early-life factors on attack frequency and treatment resistance in children with PFAPA.

Methods

Eighty-one children with PFAPA (diagnosed April 2021–2024) were retrospectively evaluated and divided into persistent and non-persistent (self-limiting) groups. Persistent PFAPA was defined as a < 50% reduction in attack frequency since diagnosis, having undergone tonsillectomy for PFAPA, or experiencing ongoing attacks beyond 7 years of age.

Results

The study included 81 PFAPA patients (female-to-male ratio 0.9; median age 76 months). The patients were divided into two groups: 28 persistent (34.6%) and 53 non-persistent (65.4%). Persistent patients had more frequent attacks (median 6 vs. 1 per year, p < 0.001) of shorter duration (median 3 vs. 4 days, p = 0.003), with less cervical lymphadenopathy (10.7% vs. 30.2%, p = 0.049) and abdominal pain (32.1% vs. 56.6%, p = 0.036). Preterm birth and pacifier use were less common in persistent patients (7.1% vs. 26.4%, p = 0.038; 25% vs. 52.8%, p = 0.016). In multivariate analysis, abdominal pain, preterm birth, and pacifier use independently predicted non-persistence, whereas colchicine use was non-significant.

Conclusion

Early-life factors, including preterm birth and pacifier use, are associated with a self-limiting PFAPA phenotype.

Impact

This study reveals that specific early-life factors—namely preterm birth and pacifier use—are independently associated with a milder, non-persistent PFAPA course.

By identifying these perinatal and environmental influences, the research shifts the focus from purely clinical symptoms to the role of early immune maturation in disease trajectory.

These findings provide clinicians with new tools to predict disease persistence, enabling more personalized management and better-informed parental counseling.

The association between early-life exposures and PFAPA persistence offers a novel perspective on the pathogenesis of autoinflammatory conditions in children.