Periodic fevers
Question:
3/26/2020
I have 10 year-old white male with periodic fevers > 5 years. Extensive workup benign. Suspicion of PFAPA. His Fevers run 102-104 range and can last 7-14 days. No arthralgias or conjunctivitis or rash during these episodes. Fevers don't respond to NSAID or Tylenol. I haven't tried Prednisone, Cimetidine or Singulair. Past Immune w/u normal: Normal CBC, Ig's, T & B cell panel; Neg. ANA/RF; Normal Complements; Normal Aldolase/CPK. He recently had right middle lobe pneumonia treated with Z pak and Cefdinir-RML PNA resolved. Repeat autoimmune workup during recent flare negative - CRP < 0.4. FHx benign. No FMF either. If he has another episode of pneumonia do sweat chloride test; planning to give Pneumovax then check post titers. Will do Mitogen assays (not sure if that will add any value?) What other w/u or Rx will you recommend?
Answer:
Prednisone 1-2 mg/kg is usually effective in relieving symptoms in PFAPA within a few hours if started close to fever onset. Consider a genetics consultation because current clinical criteria lack diagnostic accuracy. Genomics 2020 doi: 10.1016/j.ygeno.2020.03.012.
Please refer to the classification criteria for auto-inflammatory recurrent fevers
Ann Rheum Dis 2019;78:1025-32
Please review the diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS)
Ann Rheum Dis 2017;76:942-7
And finally review the evolving frontiers in the treatment of PFAPA syndrome
IMAJ 2017;19:444-7
Eric Macy, MD, MS, FAAAAI