OC onnor PFAPA Case Report USAFP 7 by OZm3HLo6

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Cormac J. O’Connor
        MD, LT, MC, USN
Naval Hospital Camp Pendleton, CA
• Fever is the presenting complaint in
  30-50% of pediatric acute visits

• Most “recurrent fevers” are actually
  superimposed viral infections

• In periodic fever cases in the U.S.,
  PFAPA is the most common cause
•   Periodic
•   Fever
•   Aphthous ulcers
•   Pharyngitis
•   Adenopathy
           Key Points
 – 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
        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

• 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
• Medline and Google searches
  using the keywords Recurrent,
  Pediatric and Fever identified in the
  literature similarities between this
  case and the PFAPA syndrome
• 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.)
• Onset typically 2-5yo

• Usually resolves by 8yo

• Male > female

• No known ethnic predilection
  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
       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?
  – 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
• Prednisone
  – 2mg/kg/d x 1-2 doses

• Tonsillectomy

• Cimetidine
  – 150mg/d
  When to Suspect and What to
• 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
  – Cyclic Neutropenia

• Recurrent, non-periodic, fever
  – recurrent tonsillitis, EBV, HBV, FMF,
• 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
Differentiating PFAPA

  Thomas, K. et al. Periodic fever syndrome in children. J Pediatr 135 (1): 15-21.

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