article · Children
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis, known as PFAPA syndrome, is the most common periodic fever syndrome affecting children. The condition manifests as regular, stereotyped episodes lasting between three and seven days and recurring every two to eight weeks. Manifestations include fever, pharyngitis, swollen neck lymph nodes, and mouth ulcers. Although the exact cause remains incompletely understood, the syndrome involves immune dysregulation linked to multiple genetic susceptibility variants. Diagnosis is primarily based on clinical evaluation, with targeted genetic testing reserved for selected cases that overlap with monogenic autoinflammatory conditions. Therapeutic management aims to minimise the frequency and severity of episodes. Acute attacks are treated with antipyretics, corticosteroids, or anti-interleukin-1 agents, whilst prevention involves colchicine, cimetidine, or tonsillectomy, alongside supportive options such as probiotics and vitamin D.
Recurrent fevers in children often lead to diagnostic confusion and the inappropriate prescribing of antibiotics. Clarifying the clinical presentation, underlying immune mechanisms, and established management options for PFAPA syndrome enables paediatric practitioners to identify the disorder earlier. This ensures children receive effective, targeted symptom relief and avoids repeated treatments that do not resolve the underlying inflammatory episodes.
The abstract does not indicate a direct commercialisation pathway, as it summarises a review of clinical literature. It highlights applied and currently used interventions, such as targeted genetic tests, anti-interleukin-1 therapies, and adjunctive supplements like probiotics, which serve paediatricians and clinical specialists in managing autoinflammatory diseases.
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Background/Objectives: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequently encountered periodic fever syndrome in pediatric populations. It is characterized by recurrent, stereotyped episodes of fever accompanied by pharyngitis, cervical lymphadenopathy, and aphthous stomatitis, sometimes with other additional nonspecific symptoms. Episodes usually last 3–7 days and recur at regular intervals of approximately 2–8 weeks. The etiopathogenesis of PFAPA remains incompletely understood. Accumulating evidence supports a central role of immune dysregulation with multiple genetic variants that contribute to disease susceptibility. Diagnosis is primarily based on clinical evaluation, although targeted genetic testing may be considered in selected cases particularly when overlapping with other monogenic autoinflammatory disorders. Management of PFAPA aims to decrease the frequency and intensity of febrile episodes. Acute flares are managed with antipyretics, corticosteroids, or, in selected cases, anti-interleukin-1 (IL-1) therapy, while preventive strategies include colchicine, cimetidine, or tonsillectomy. Adjunctive approaches such as vitamin D supplementation, probiotics, and other immunomodulatory interventions have also been investigated. This updated review summarizes current insights into the pathogenesis and therapeutic approaches of PFAPA. Methods: This narrative review was conducted using PubMed, and Scopus to identify English-language publications from 2010 to 2026 addressing PFAPA. Conclusions: Pediatricians should consider PFAPA in children with recurrent fevers and oropharyngeal symptoms, especially when standard treatments fail, to ensure proper management and avoid unnecessary antibiotic use.
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DOI: 10.3390/children13091167
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