Periodic Fever, Aphthae, Pharyngitis and Cervical Adenitis. Our experience in the management of PFAPA syndrome
DOI:
https://doi.org/10.34631/sporl.592Keywords:
PFAPA syndrome, Marshall´s syndrome, periodic fever, tonsillectomy, corticoid therapyAbstract
Aim: To analyze our experience in the management of PFAPA syndrome (Periodic Fever, Adenopathy, Pharyngitis and Aphthae) and literature review. Patients and methods: observational, prospective, descriptive study of 8 patients younger than 15 years, diagnosed with PFAPA syndrome according to Thomas´ criteria, between August 2014 and January 2015. Epidemiological, clinical and laboratory findings are discussed. Results: Median age at onset of symptoms 3.4 years; mean lag time from onset of symptoms and diagnosis 3.1 years. 100% presented fever and pharyngitis, 87.5% cervical adenitis and 67.5% aphthous stomatitis. Mean symptom-free interval 27.1 days. Good response to single dose of corticosteroids in 100%. One patient undergoing tonsillectomy. Conclusions: PFAPA syndrome is a rare benign disease, whose knowledge allows early diagnosis and appropriate treatment, providing a better quality of life for patients and optimization of diagnostic and therapeutic resources.
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