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PFAPA Syndrome

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PFAPA Syndrome, Marshall's Syndrome, PFAPA

  • See Also
  1. Recurrent Fever
  2. Aphthous Ulcer
  3. Pharyngitis
  4. Pharyngitis Causes
  5. Dysphagia
  6. Tonsillectomy Indications
  • Epidemiology
  1. Most common in age under 5 years
  2. Most common cause of Recurrent Fever in children
  • Signs
  • (PFAPA acronym)
  1. Periodic Fever (PF)
    1. High fever lasting 3-6 days
    2. Recurs every 3-4 weeks
  2. Aphthous Ulcer (A)
    1. Small lesions which are often asymptomatic
  3. Pharyngitis (P)
  4. Cervical Lymphadenopathy (A)
  5. Child is well between monthly exacerbations
  • Differential diagnosis
  1. See Recurrent Fever
  2. See Aphthous Ulcer
  3. See Pharyngitis
  4. Cyclic Neutropenia
  5. Behcet's Disease
  • Management
  1. Prednisone 2 mg/kg may be considered at start of symptoms
  2. Agents that have been tried for prophylaxis
    1. Colchicine
    2. Cimetidine
  3. Tonsillectomy effective in small studies
    1. No recurrence after 10 months
    2. Berlucchi (2003) Ann Otol Rhinol Laryngol 112:365-9 [PubMed]
    3. Galanakis (2002) Arch Dis Child 86(6):434-5 [PubMed]
  • References
  1. John (2002) Pediatr Infect Dis J 21:1071-80 [PubMed]
  2. Vanoni (2016) Pediatr Rheumatol Online J 14(1):38 +PMID:27349388 [PubMed]