Journal article
Physicians' perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome
Rheumatology international, v 37(6), pp 883-889
01 Jun 2017
PMID: 28271158
Featured in Collection : UN Sustainable Development Goals @ Drexel
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.
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Details
- Title
- Physicians' perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome
- Creators
- Kalpana Manthiram - Vanderbilt UniversitySuzanne C. Li - Hackensack University Medical CenterJonathan S. Hausmann - Beth Israel Deaconess Medical CenterGil Amarilyo - Tel Aviv UniversityKaryl Barron - National Institutes of HealthHanna Kim - National Institutes of HealthSimona Nativ - Overlook Medical CenterGeraldina Lionetti - University of California, San FranciscoAndrew Zeft - Cleveland ClinicDonald Goldsmith - Drexel UniversityDavid Kimberlin - University of Alabama at BirminghamKathryn Edwards - Vanderbilt UniversityFatma Dedeoglu - Boston Children's HospitalSivia Lapidus - Overlook Medical CenterChildhood Arthrit Rheumatology Res
- Publication Details
- Rheumatology international, v 37(6), pp 883-889
- Publisher
- Springer Nature
- Number of pages
- 7
- Grant note
- RC2AR058934 / National Institute of Arthritis and Musculoskeletal and Skin Diseases; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) Duke Clinical Research Institute Arthritis Foundation UL1 TR000445 / Vanderbilt Institute for Clinical and Translational Research from NCATS/NIH; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Center for Advancing Translational Sciences (NCATS) Novartis Friends of CARRA 1T32HD060554-05 / Conducting Child Health Care Research in Vulnerable Populations training Grants from Vanderbilt University School of Medicine 1T32AI095202-01 / Childhood Infection Research Program from Vanderbilt University School of Medicine ZIAHG200371 / NATIONAL HUMAN GENOME RESEARCH INSTITUTE; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Human Genome Research Institute (NHGRI)
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics
- Web of Science ID
- WOS:000401632400004
- Scopus ID
- 2-s2.0-85014579945
- Other Identifier
- 991019168242804721
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- Collaboration types
- Domestic collaboration
- International collaboration
- Web of Science research areas
- Rheumatology