Antonio Vitale1, Ida Orlando1, Giuseppe Lopalco2, Giacomo Emmi3, Marco Cattalini4, Bruno Frediani1, Mauro Galeazzi1, Florenzo Iannone2, Donato Rigante5, Luca Cantarini6. 1. Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy. 2. Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari Aldo Moro, Bari, Italy. 3. Department of Experimental and Clinical Medicine, University of Florence, Italy. 4. Paediatric Clinic, University of Brescia and Spedali Civili di Brescia, Italy. 5. Institute of Paediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario "A. Gemelli", Rome, Italy. 6. Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy. cantariniluca@hotmail.com.
Abstract
OBJECTIVES: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a non-Mendelian autoinflammatory disorder until now considered to be specifically limited to paediatric age. Recently, an increasing number of reports seems to suggest that PFAPA syndrome, diagnosed by the Marshall criteria revised by Thomas et al., can also affect adults. METHODS: The Marshall/Thomas criteria have been applied to 989 adult patients presenting for recurrent fever episodes: all patients enrolled were reviewed for demographic, clinical, and therapeutic data. Infectious, neoplastic, autoimmune and other autoinflammatory diseases were ruled out. RESULTS: We identified 30 adult patients (19 males, 11 females) with a suspected PFAPA syndrome: their mean age at disease onset was 33.75±14.01 years, mean age at diagnosis 39.1±14.39 years, and mean body temperature peak 39.5±0.7°C. In addition, the mean frequency of febrile episodes was 11.58±8.97 per year. More precisely, patients complained of pharyngitis (77%), cervical adenitis (73%), asthenia (63%), arthralgia (67%), oral aphthosis (50%), myalgia (54%), cephalalgia (43%), abdominal pain (27%), nausea/vomiting (17%), periorbital pain (17%), and arthritis (10%). Six out of 30 (20%) patients had suffered from PFAPA syndrome also during childhood, and the disease had reappeared in adulthood. CONCLUSIONS: We provide the largest monocentric cohort of patients diagnosed with a suspected PFAPA syndrome in adulthood confirming that this syndrome can occur also during adulthood; moreover, due to the medical history of our patients and based on our experience, PFAPA syndrome might relapse during adulthood after a temporary remission reached in the course of paediatric age.
OBJECTIVES: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a non-Mendelian autoinflammatory disorder until now considered to be specifically limited to paediatric age. Recently, an increasing number of reports seems to suggest that PFAPA syndrome, diagnosed by the Marshall criteria revised by Thomas et al., can also affect adults. METHODS: The Marshall/Thomas criteria have been applied to 989 adult patients presenting for recurrent fever episodes: all patients enrolled were reviewed for demographic, clinical, and therapeutic data. Infectious, neoplastic, autoimmune and other autoinflammatory diseases were ruled out. RESULTS: We identified 30 adult patients (19 males, 11 females) with a suspected PFAPA syndrome: their mean age at disease onset was 33.75±14.01 years, mean age at diagnosis 39.1±14.39 years, and mean body temperature peak 39.5±0.7°C. In addition, the mean frequency of febrile episodes was 11.58±8.97 per year. More precisely, patients complained of pharyngitis (77%), cervical adenitis (73%), asthenia (63%), arthralgia (67%), oral aphthosis (50%), myalgia (54%), cephalalgia (43%), abdominal pain (27%), nausea/vomiting (17%), periorbital pain (17%), and arthritis (10%). Six out of 30 (20%) patients had suffered from PFAPA syndrome also during childhood, and the disease had reappeared in adulthood. CONCLUSIONS: We provide the largest monocentric cohort of patients diagnosed with a suspected PFAPA syndrome in adulthood confirming that this syndrome can occur also during adulthood; moreover, due to the medical history of our patients and based on our experience, PFAPA syndrome might relapse during adulthood after a temporary remission reached in the course of paediatric age.
Authors: Francesca Della Casa; Antonio Vitale; Marco Cattalini; Francesco La Torre; Giovanna Capozio; Emanuela Del Giudice; Maria Cristina Maggio; Giovanni Conti; Maria Alessio; Benson Ogunjimi; Gaafar Ragab; Giacomo Emmi; Emma Aragona; Teresa Giani; Giuseppe Lopalco; Paola Parronchi; Farhad Shahram; Elena Verrecchia; Francesca Ricci; Fabio Cardinale; Silvia Di Noi; Rossana Nuzzolese; Riccardo Lubrano; Serena Patroniti; Roberta Naddei; Vito Sabato; Mohamed A Hussein; Laura Dotta; Violetta Mastrorilli; Stefano Gentileschi; Abdurrahman Tufan; Valeria Caggiano; Mohamed Tharwat Hegazy; Jurgen Sota; Ibrahim A Almaghlouth; Amr Ibrahim; Ewa Wiȩsik-Szewczyk; Burcugul Ozkiziltas; Salvatore Grosso; Micol Frassi; Maria Tarsia; Rosa Maria R Pereira; Maged Taymour; Carla Gaggiano; Sergio Colella; Claudia Fabiani; Maria Morrone; Piero Ruscitti; Bruno Frediani; Veronica Spedicato; Henrique A Mayrink Giardini; Alberto Balistreri; Donato Rigante; Luca Cantarini Journal: Front Pediatr Date: 2022-07-22 Impact factor: 3.569
Authors: Karin Rydenman; Hanna Fjeld; Josefine Hätting; Stefan Berg; Anders Fasth; Per Wekell Journal: Pediatr Rheumatol Online J Date: 2022-09-15 Impact factor: 3.413