title: PFAPA syndrome and Behçet's disease: a comparison of two medical entities based on the clinical interviews performed by three different specialists.
abstract: The pediatric syndrome characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) and adult Behçet's disease share some clinical manifestations and are both polygenic autoinflammatory disorders with interleukin-1β showing to play a pivotal role. However, the diagnosis is mostly clinical and we hypothesize that specific criteria may be addressed differently by different physicians. To determine the diagnostic variability, we compared the answers of 80 patients with a definite diagnosis of Behçet's disease (age 42.1 ± 13.7 years) obtained by separate telephone interviews conducted by a rheumatologist, a pediatrician, and an internist working largely in the field of autoinflammatory disorders. Questions were related to the age of symptom onset, the occurrence of recurrent fevers during childhood, and the association with oral aphthosis, cervical adenitis and/or pharyngitis, previous treatments, possible growth impairment, the time lapse between PFAPA-like symptoms and the onset of Behçet's disease, and the occurrence of Behçet-related manifestation during childhood. The rheumatologist identified 30 % of patients with Behçet's disease fulfilling PFAPA syndrome diagnostic criteria, compared to the pediatrician and the internist identifying 10 and 7.5 %, respectively. Most of the patients suffered from recurrent oral aphthosis in childhood also without fever (50, 39, and 48 % with each interviewer), yet no patient fulfilled the Behçet's disease diagnostic criteria. Our data suggest that physician awareness and expertise are central to the diagnosis of autoinflammatory disorders through an accurate collection of the medical history.
authors: 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, 53100, Siena, Italy. cantariniluca@hotmail.com.
authors: Luca
authors: L
authors: Cantarini
authors: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors: Antonio
authors: A
authors: Vitale
authors: Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy.
authors: Giulia
authors: G
authors: Bersani
authors: Rheumatology Service, Hospital Regional Universitario Carlos Haya, University of Màlaga, Màlaga, Spain.
authors: Laura Martin
authors: LM
authors: Nieves
authors: Pediatric Clinic, University of Brescia and Spedali Civili di Brescia, Brescia, Italy.
authors: Marco
authors: M
authors: Cattalini
authors: Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy.
authors: Giuseppe
authors: G
authors: Lopalco
authors: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.|Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Padua, Padua, Italy.
authors: Francesco
authors: F
authors: Caso
authors: Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, Naples, Italy.
authors: Luisa
authors: L
authors: Costa
authors: Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy.
authors: Florenzo
authors: F
authors: Iannone
authors: Interdisciplinary Department of Medicine, Rheumatology Unit, Policlinic Hospital, University of Bari, Bari, Italy.
authors: Giovanni
authors: G
authors: Lapadula
authors: Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
authors: Mauro
authors: M
authors: Galeazzi
authors: Division of Rheumatology, Allergy, and Clinical Immunology, University of CA, Davis, CA, USA.
authors: Angela
authors: A
authors: Ceribelli
authors: Division of Rheumatology, Allergy, and Clinical Immunology, University of CA, Davis, CA, USA.
authors: Enrico
authors: E
authors: Brunetta
authors: Division of Rheumatology, Allergy, and Clinical Immunology, University of CA, Davis, CA, USA.|Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milan, Italy.
authors: Carlo
authors: C
authors: Selmi
authors: Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy.
authors: Donato
authors: D
authors: Rigante
mesh_terms: D000293:Adolescent; D000328:Adult; D001528:Behcet Syndrome / Q000453:epidemiology*; D005334:Fever / Q000453:epidemiology; D006801:Humans; D007388:Internal Medicine / Q000706:statistics & numerical data; D007407:Interviews as Topic; D007558:Italy / Q000453:epidemiology; D008199:Lymphadenitis / Q000453:epidemiology; D008875:Middle Aged; D010372:Pediatrics / Q000706:statistics & numerical data; D010612:Pharyngitis / Q000453:epidemiology; D012219:Rheumatology / Q000706:statistics & numerical data; D013280:Stomatitis / Q000453:epidemiology; D013577:Syndrome; D055815:Young Adult
keywords: Autoinflammation; Behçet’s disease; Interleukin-1; PFAPA syndrome