title: Physicians' perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.
abstract: 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.
authors.affiliation: Vanderbilt University School of Medicine, Nashville, TN, USA.
authors.forename: Kalpana
authors.initials: K
authors.lastname: Manthiram
authors.affiliation: Hackensack University Medical Center, Hackensack, NJ, USA.
authors.forename: Suzanne C
authors.initials: SC
authors.lastname: Li
authors.affiliation: Boston Children's Hospital, Boston, MA, USA.|Beth Israel Deaconess Medical Center, Boston, MA, USA.
authors.forename: Jonathan S
authors.initials: JS
authors.lastname: Hausmann
authors.affiliation: Schneider Children's Medical Center of Israel, Petach Tikva, Israel.|Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Gil
authors.initials: G
authors.lastname: Amarilyo
authors.affiliation: National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Karyl
authors.initials: K
authors.lastname: Barron
authors.affiliation: National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
authors.forename: Hanna
authors.initials: H
authors.lastname: Kim
authors.affiliation: Pediatric Rheumatology, Goryeb Children's Hospital, Atlantic Health System, Morristown Medical Center/Overlook Hospital, Morristown, NJ, USA.
authors.forename: Simona
authors.initials: S
authors.lastname: Nativ
authors.affiliation: University of California, San Francisco, San Francisco, CA, USA.
authors.forename: Geraldina
authors.initials: G
authors.lastname: Lionetti
authors.affiliation: The Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Andrew
authors.initials: A
authors.lastname: Zeft
authors.affiliation: Drexel University College of Medicine, Philadelphia, PA, USA.
authors.forename: Donald
authors.initials: D
authors.lastname: Goldsmith
authors.affiliation: Pediatric Infectious Diseases Society, Arlington, VA, USA.|University of Alabama at Birmingham, Birmingham, AL, USA.
authors.forename: David
authors.initials: D
authors.lastname: Kimberlin
authors.affiliation: Vanderbilt University School of Medicine, Nashville, TN, USA.
authors.forename: Kathryn
authors.initials: K
authors.lastname: Edwards
authors.affiliation: Boston Children's Hospital, Boston, MA, USA.
authors.forename: Fatma
authors.initials: F
authors.lastname: Dedeoglu
authors.affiliation: Pediatric Rheumatology, Goryeb Children's Hospital, Atlantic Health System, Morristown Medical Center/Overlook Hospital, Morristown, NJ, USA. sivia.lapidus@atlantichealth.org.
authors.forename: Sivia
authors.initials: S
authors.lastname: Lapidus
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D058633:Antipyretics / Q000627:therapeutic use; D001291:Attitude of Health Personnel; D005334:Fever / Q000175:diagnosis* / Q000628:therapy*; D019983:Guideline Adherence; D019538:Health Care Surveys; D007722:Health Knowledge, Attitudes, Practice; D006635:Histamine H2 Antagonists / Q000627:therapeutic use; D006801:Humans; D008199:Lymphadenitis / Q000175:diagnosis* / Q000628:therapy*; D000072143:Pediatricians / Q000523:psychology*; D010612:Pharyngitis / Q000175:diagnosis* / Q000628:therapy*; D017410:Practice Guidelines as Topic; D010818:Practice Patterns, Physicians'; D011237:Predictive Value of Tests; D000072140:Rheumatologists / Q000523:psychology*; D013038:Specialization; D013281:Stomatitis, Aphthous / Q000175:diagnosis* / Q000628:therapy*; D014068:Tonsillectomy; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D058633:Antipyretics; D006635:Histamine H2 Antagonists
keywords: Autoinflammatory syndrome; PFAPA; Periodic fever; Physician practice patterns