1065. Characteristics and Management of Children with PFAPA Syndrome – A Retrospective Study of 52 Patients
Session: Poster Abstract Session: Herpesviruses, HPV, and Other Viruses
Friday, October 9, 2015
Room: Poster Hall
  • IUSMposter_3X6 - PFAPA Chehab, Hiba.pdf (144.0 kB)
  • Background: Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) syndrome was first described by Marshall et al in 1987. We studied the characteristics and management of 52 children with PFAPA.

    Methods: We reviewed medical records of 385 patients assigned ICD-9 codes for recurrent or periodic fevers after being seen in the Pediatric Infectious Disease (PID) clinic between 2007 and 2013. 52 patients were diagnosed with PFAPA. We studied the demographics, clinical characterisitics, and management in these 52 patients.

    Results: 50% (n=26) of patients were coded as periodic fever on their initial visit. 50% (n=26) were female. 92% (n=48) were white. 71% (n=37) had commercial insurance or were self-payers. Mean and median age at onset of symptoms was 29 and 20 months respectively. Mean and median age at first visit to the PID clinic were 47 and 40 months respectively. Fever cycle had a mean and median duration of 5.2 and 5 days, a mean and median frequency of 3.3 and 3 weeks, and a mean and median T-max of 104.3 and 104 degrees F respectively. Most commonly documented symptoms were anorexia (67%, n=35), fatigue (65%, n=34), sore throat (63%, n=33), and lymphadenopathy (56%, n=29). Mouth sores were reported in 31% (n=16) of patients. The majority (69% (n=36)) had normal physical exam at the time of their visit. Lab evaluation included ESR and/or CRP (88%, n=46), CBC (98%, n=51), CMP or BMP (85%, n=44), and urinalysis (83%, n=43). Mean and median ESR were 26.3 and 18.0 respectively. All ESR values > 30 were measured during the first 14 days since onset of a given fever episode, except for one patient who had an ESR of 45 (dropping from 58) on day 18. All patients were prescribed steroids, 3.8% (n=2) refused steroids. All patients, except one who had previous tonsillectomy, were offered referral for tonsillectomy. 13.5% (n=7) patients declined surgery referral.

    Conclusion: PFAPA patients in our PID clinic report similar symptoms as previously described in existing literature. Most patients have normal physical exams during their evaluation and anorexia is the most common reported symptom during episodes. There is > 18 months between onset of symptoms and first clinic visit. Once PFAPA diagnosis is made, tonsillectomy referral is part of routine management.

    Hiba Chehab, MD, Pediatrics, Indiana University School of Medicine, Indianapolis, IN, Annette Foti Childress, MD, Pediatrics, Ryan White Center for Pediatric Infectious Disease, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN and S. Maria E. Finnell, MD, MS, Pediatrics, Ryan White Center for Pediatric Infectious Disease, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN; Pediatrics, Children’s Health Services Research, Indiana University School of Medicine, Indianapolis, IN


    H. Chehab, None

    A. Foti Childress, None

    S. M. E. Finnell, None

    Findings in the abstracts are embargoed until 12:01 a.m. PDT, Wednesday Oct. 7th with the exception of research findings presented at the IDWeek press conferences.