title: A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome.
abstract: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a mysterious disorder characterized by periodically recurrent fevers, oropharyngeal inflammation, and adenitis, which mainly affects children, though in very recent times, it has been also recognized in adulthood. We enrolled 115 unrelated pediatric and adult patients with history of periodic fevers who fulfilled the current diagnostic criteria for PFAPA syndrome in three Italian referral centers and highlighted differences between children and adults. Eighty-five children and 30 adults were evaluated: the frequency of flares was significantly higher in pediatric cases, while febrile attack duration was significantly longer in adults. Clockwork periodicity of fever and recurrent pharyngitis were more frequently observed in childhood, but no differences were identified for aphthosis and cervical adenopathy. Conversely, joint symptoms, myalgia, headache, fatigue, ocular signs, and rashes were more common in adults. The simultaneous occurrence of two or three cardinal PFAPA signs did not show any statistical difference between the groups, while the occurrence of only one cardinal manifestation was more frequent in adults. Corticosteroids were effective in 98.82 % of children and 88.2 % of adults. Tonsillectomy was rarely performed, resulting effective in only two patients. Our data illustrate the clinical overlap between pediatric and adult cases of PFAPA syndrome. Adults are characterized by a wider repertoire of inflammatory signs, suggesting that onset in adulthood might leave the disease misdiagnosed. Clinicians, not only pediatricians, should take into account this clinical entity in every patient of whatever age suffering from recurrent fevers of unknown origin.
authors.affiliation: Institute of Pediatrics, Periodic Fever Research Center, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario "A. Gemelli", Rome, Italy.
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Vitale
authors.affiliation: Institute of Pediatrics, Periodic Fever Research Center, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario "A. Gemelli", Rome, Italy.
authors.forename: Marco Francesco
authors.initials: MF
authors.lastname: Natale
authors.affiliation: Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari, Bari, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lopalco
authors.affiliation: Institute of Pediatrics, Periodic Fever Research Center, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario "A. Gemelli", Rome, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Andreozzi
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors.forename: Bruno
authors.initials: B
authors.lastname: Frediani
authors.affiliation: Institute of Pediatrics, Periodic Fever Research Center, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario "A. Gemelli", Rome, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: D'Errico
authors.affiliation: Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari, Bari, Italy.
authors.forename: Florenzo
authors.initials: F
authors.lastname: Iannone
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy. cantariniluca@hotmail.com.|Rheumatology Unit, Policlinico "Le Scotte", University of Siena, viale Bracci 1, Siena, 53100, Italy. cantariniluca@hotmail.com.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D002648:Child; D005260:Female; D005334:Fever; D006801:Humans; D007249:Inflammation; D008199:Lymphadenitis / Q000150:complications / Q000175:diagnosis*; D008206:Lymphatic Diseases / Q000150:complications / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D010612:Pharyngitis / Q000150:complications / Q000175:diagnosis*; D010641:Phenotype; D011241:Prednisone / Q000008:administration & dosage; D012008:Recurrence; D013281:Stomatitis, Aphthous / Q000150:complications / Q000175:diagnosis*; D013577:Syndrome; D014068:Tonsillectomy / Q000379:methods
chemical_list: D000305:Adrenal Cortex Hormones; D011241:Prednisone
keywords: Adulthood; Autoinflammatory disorders; Childhood; PFAPA syndrome; Prednisone; Tonsillectomy