P2.263: When It’s Not Streptococcus: A Missed Diagnosis in Recurrent Pediatric Fever and Pharyngitis

Introduction: Recurrent fever and pharyngitis are among the most common reasons for pediatric clinic visits, often attributed to viral or streptococcal infections. Yet in a subset of children, these symptoms may follow a periodic pattern and fail to respond to standard treatments, suggesting alternative etiologies. This case highlights PFAPA syndrome—Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis—an underdiagnosed, autoinflammatory condition that closely mimics recurrent infections. Prompt recognition of PFAPA can prevent repeated antibiotic exposure, reduce healthcare utilization, and improve outcomes through targeted therapies such as corticosteroids or, in select cases, tonsillectomy.

Case Description: A 7-year-old male presented to the outpatient clinic with a low grade fever (100.3°F), sore throat, and aphthous ulcers. His parents reported recurrent febrile episodes with similar symptoms occurring every 2–4 weeks over the preceding several months. Review of systems was negative for recurrent otitis media, sinus infections, gastrointestinal complaints, fungal infections, or other signs suggestive of underlying immunodeficiency. On physical exam, the patient appeared ill and febrile with visible aphthous ulcers and tender anterior cervical lymphadenopathy.

Repeat throat cultures continued to show GAS positivity, raising concern for a GAS carrier state. Given the periodic nature of his symptoms and lack of sustained improvement with antibiotics, the differential was broadened to include PFAPA syndrome. A single dose of prednisolone (1 mg/kg) led to complete symptom resolution by the next visit. PFAPA was distinguished from other conditions by its clockwork periodicity, absence of systemic inflammation between episodes, and rapid resolution with corticosteroids. He later underwent tonsillectomy and adenoidectomy to reduce the risk of recurrence.

Discussion: PFAPA syndrome is frequently underrecognized, resulting in delayed diagnosis and unnecessary interventions. A prompt clinical response to a single dose of corticosteroids (1 mg/kg) strongly supports the diagnosis. In contrast to infectious causes, PFAPA follows a clockwork-like periodicity, with predictable symptom recurrence and normal health between flares. Early recognition is essential to reduce antibiotic exposure, healthcare costs, and diagnostic uncertainty. Although tonsillectomy may lead to long-term remission in approximately 80% of cases, recent studies suggest comparable efficacy between surgical and steroid-based treatment. Given PFAPA’s self-limiting nature, the risks and costs of surgery should be weighed against the high response rate to corticosteroids. This case illustrates the value of pattern recognition, thoughtful diagnostic reasoning, and shared decision-making in pediatric care.

Conclusion: PFAPA should be considered in children with recurrent febrile episodes that follow a stereotyped pattern and do not respond to conventional antibiotics. Early diagnosis reduces unnecessary treatments and healthcare costs while allowing effective management with corticosteroids or, when appropriate, tonsillectomy.