Physicians' perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome

Rheumatol Int. 2017 Jun;37(6):883-889. doi: 10.1007/s00296-017-3688-3. Epub 2017 Mar 7.


To assess the practice patterns of pediatric rheumatology and infectious diseases subspecialists in the diagnosis and treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. An online survey assessing diagnostic and treatment approaches was sent to 424 members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) and 980 members of the Pediatric Infectious Disease Society (PIDS). 277 physicians (123 from CARRA and 154 from PIDS representing 21% of the total membership) completed the survey. To diagnose PFAPA, most respondents agreed that patients must have the following features of the diagnostic criteria: stereotypical fever episodes (95%), asymptomatic intervals between episodes (93%), and normal growth and development (81%). However, 71% of the respondents did not require age of onset <5 years, 33% did not require regular intervals between episodes, and 79% did not require the concomitant signs of aphthous stomatitis, adenitis, or pharyngitis during episodes as long as episodes were regular. Over half (58%) considered episode resolution with steroids to be diagnostic of PFAPA. Corticosteroids, antipyretics, tonsillectomy, and cimetidine were the most commonly prescribed treatments, while steroids and tonsillectomy were most effective. Subspecialists in pediatric rheumatology and infectious diseases showed limited adherence to the complete published criteria for diagnosing PFAPA suggesting heterogeneity in the characteristics of patients diagnosed with the disorder. These findings emphasize the need to develop consensus diagnostic and treatment guidelines in well-characterized patient populations.

Keywords: Autoinflammatory syndrome; PFAPA; Periodic fever; Physician practice patterns.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Antipyretics / therapeutic use
  • Attitude of Health Personnel*
  • Fever / diagnosis*
  • Fever / therapy*
  • Guideline Adherence
  • Health Care Surveys
  • Health Knowledge, Attitudes, Practice
  • Histamine H2 Antagonists / therapeutic use
  • Humans
  • Lymphadenitis / diagnosis*
  • Lymphadenitis / therapy*
  • Pediatricians / psychology*
  • Pharyngitis / diagnosis*
  • Pharyngitis / therapy*
  • Practice Guidelines as Topic
  • Practice Patterns, Physicians'
  • Predictive Value of Tests
  • Rheumatologists / psychology*
  • Specialization
  • Stomatitis, Aphthous / diagnosis*
  • Stomatitis, Aphthous / therapy*
  • Tonsillectomy
  • Treatment Outcome


  • Adrenal Cortex Hormones
  • Antipyretics
  • Histamine H2 Antagonists