Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: main features and an algorithm for clinical practice

Rheumatology International
Ezgi Deniz Batu

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome of early childhood with increasing number of adult-onset cases. Although it is a self-limited disease, it may negatively affect the quality of life. The aim of this review is to present a detailed analysis of PFAPA syndrome and an algorithm for diagnosis, therapeutic options, and evaluation of outcome. A comprehensive literature search was conducted through the Cochrane Library, Scopus, and MEDLINE/PubMed databases. The main topics covered are the epidemiology, clinical manifestations, diagnosis, differential diagnosis, etiopathogenesis, genetics, management, disease course and prognosis, disease in adults, unsolved issues, and unmet needs in PFAPA. The diagnosis of PFAPA is mainly based on clinical classification criteria. The most relevant hypothesis for pathogenesis is that dysregulated immune system in a genetically predisposed individual responds to a yet unidentified trigger in an exaggerated way. The pedigree analyses suggest a genetic background for the disease with an autosomal dominant pattern of inheritance. For management, single-dose corticosteroids during attacks and tonsillectomy remain the most ...Continue Reading

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Citations

Sep 9, 2020·International Journal of Pediatric Otorhinolaryngology·Ali Karayağmurlu, İsmail Aytaç
May 12, 2021·Pediatric Rheumatology Online Journal·Martina GirardelliAlberto Tommasini
May 20, 2021·Clinical and Experimental Immunology·Giorgio Costagliola, Rita Consolini
Aug 26, 2021·World Journal of Otorhinolaryngology - Head and Neck Surgery·Alicia WangGreg R Licameli

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