Mil Med. 2026 Mar 27:usag138. doi: 10.1093/milmed/usag138. Online ahead of print.
ABSTRACT
INTRODUCTION: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) is a rare, self-resolving, under-recognized autoinflammatory condition impacting young children. There is limited management and outcome data on PFAPA. Our study aims to describe the diagnostic and treatment approach, family impact, and outcomes of children with PFAPA from the perspective of their parents.
MATERIALS AND METHODS: We performed a survey-based case series study targeted toward parents of children diagnosed with PFAPA. Department of Defense beneficiaries diagnosed with PFAPA between ages 0 and 18 years and cared for by a Military Pediatric Infectious Disease physician at Naval Medical Center Portsmouth or Walter Reed National Capital Consortium between 2012 and 2023 were included. Parents of these patients were contacted via telephone and invited to participate in the online anonymous survey covering PFAPA diagnostic timeline, management strategies, clinical outcomes, and the impact on the patient’s family. Quantitative data from the survey was analyzed using descriptive statistics, and qualitative data from free text responses underwent manual thematic analysis.
RESULTS: Twenty-two children were identified with PFAPA, and 17 parents were successfully contacted and invited to participate. Of these, 10 parents completed the survey. For eight (80%) patients, a PFAPA diagnosis required more than four healthcare visits and took greater than a year from symptom onset. Seven parents reported use of abortive corticosteroids for PFAPA treatment, and four (40%) underwent tonsillectomy, resulting in immediate cessation of fever episodes for three of the four patients. At the time of the survey, 70% of children with PFAPA had experienced resolution of their fever episodes. Nearly every parent stated that PFAPA led to missed school and workdays. Half of respondents stated that PFAPA negatively impacted their child’s mental health and/or family well-being. More than half (60%) of respondents expressed feeling dismissed in their concerns and wished more providers were knowledgeable about PFAPA.
CONCLUSIONS: For most children with PFAPA, diagnosis requires more than four healthcare visits and over a year of time. PFAPA is a self-limited condition but poorly impacts families through missed school, missed workdays, and other family life disruptions. Parents of children with PFAPA want to be heard when they express concerns about frequent fever episodes and want providers to be better informed about PFAPA and available treatment options.
PMID:41896172 | DOI:10.1093/milmed/usag138
