PFAPA SYNDROME Cormac J. O’Connor MD, LT, MC, USN Naval Hospital Camp Pendleton, CA Introduction • Fever is the presenting complaint in 30-50% of pediatric acute visits • Most “recurrent fevers” are actually superimposed viral infections Introduction • In periodic fever cases in the U.S., PFAPA is the most common cause PFAPA • Periodic • Fever • Aphthous ulcers • Pharyngitis • Adenopathy Key Points • PFAPA – Fevers are brief (<5d), unheralded periodic fevers with “APA” symptoms and no other focus of disease – Wellness between fevers with normal growth, development and activity Key Points • Patients with PFAPA are completely well • Have fewer viral URI’s than their siblings Case Presentation • 2 ½-year-old boy in ER for 2 days of fever to 101F and neck tenderness, without URI symptoms • Exam, CBC and UA were normal, d/c home with “febrile illness” • Symptoms resolved fully in 3-4 days Case Presentation • Symptoms recurred every 4 weeks for the next 18 months • Review of medical, family, social, travel, and exposure histories was unremarkable • Multiple ER visits resulted in febrile illness or URI diagnoses Case Presentation • Most impressive was the speed of clinical change • Oral ulcers noted with fever • Referred to NMCSD pediatric infectious disease department Case Presentation • Febrile episodes recurred every four weeks from 2 ½ - 4 years old • He is nearly 6 years old and in full health without sequelae Discussion • Medline and Google searches using the keywords Recurrent, Pediatric and Fever identified in the literature similarities between this case and the PFAPA syndrome Discussion • Identified in 1987 by Marshall, et al – aka Marshall’s syndrome, then later PFAPA syndrome • Etiology – Infectious vs. Autoimmune unclear • Incidence – Far greater than other recurrent fevers (Cyclic neutropenia, FMF, HIDS, etc.) Discussion • Onset typically 2-5yo • Usually resolves by 8yo • Male > female • No known ethnic predilection Discussion Periodic Fever • Adenopathy – usually 3-5 days – cervical – repeats every 3-4 – usually bilateral and weeks short-lived • Aphthous ulcers • No skin, respiratory, – small, shallow, and gastrointestinal, or resolve rapidly joint involvement • Pharyngitis – with or without exudate Diagnostic Criteria • Periodic fevers onset < 5yo • Absence of URI sx including >1 of: – apthous stomatitis – cervical lymphadenitis – pharyngitis • Exclude cyclic neutropenia • Normal, asymptomatic intervals Why isn’t it Cyclic Neutropenia? • PFAPA – Onset ~ 2-5yo – Fever 3-5d q 4weeks, no neutropenia – Well intervals • Cyclic neutropenia – Onset usually during infancy – Fever 5-7d, q 21d, with ANC <200/mm3 – Associated infections Treatment • Prednisone – 2mg/kg/d x 1-2 doses • Tonsillectomy • Cimetidine – 150mg/d When to Suspect and What to Do? • In typical presentations – No concern for other dx • After 3-4 febrile episodes • One expert recommends only CBC, throat Cx, ESR, & ASO titer Differential Diagnoses • Periodic Fever Syndromes – PFAPA – Cyclic Neutropenia • Recurrent, non-periodic, fever syndromes – recurrent tonsillitis, EBV, HBV, FMF, HIDS, TRAPS, JRA Conclusion • PFAPA is the #1 cause of periodic fever in the U.S.A. • Punctual periodic fevers, with aphthous ulcers, pharyngitis, and cervical adenopathy • Completely well intervals Questions? Differentiating PFAPA Thomas, K. et al. Periodic fever syndrome in children. J Pediatr 135 (1): 15-21.
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