Ludovico Luca Sicignano1, Donato Rigante2,3, Beatrice Moccaldi1, Maria Grazia Massaro1, Stefano Delli Noci4, Isabella Patisso5, Giovanna Capozio4, Elena Verrecchia1, Raffaele Manna1,6. 1. Division of Internal Medicine, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy. 2. Department of Life Sciences and Public Health, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy. donato.rigante@unicatt.it. 3. Università Cattolica del Sacro Cuore, Rome, Italy. donato.rigante@unicatt.it. 4. Department of Life Sciences and Public Health, Rare Diseases and Periodic Fevers Research Centre, Fondazione Policlinico A. Gemelli IRCCS, Rome, Italy. 5. Pediatrics, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy. 6. Università Cattolica del Sacro Cuore, Rome, Italy.
Abstract
INTRODUCTION: Analogies or differences of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in children and adults are barely known. The aim of our study was to compare the overall characteristics of a large cohort of patients, both children and adults, diagnosed with PFAPA syndrome. METHODS: In the last decade, we identified 120 children and 63 adults with periodically recurring fevers, who fulfilled the criteria for PFAPA diagnosis. The two subcohorts were analyzed according to demographic features, clinical manifestations, laboratory data, and responses to therapies. RESULTS: The mean age of onset was 2.4 ± 1.5 and 19.7 ± 10.3 years, respectively, in children and adults, while attacks occurred every 3.8 ± 0.8 and every 4.3 ± 2.3 weeks, respectively, in children and adults. A higher prevalence of exudative pharyngitis was observed in children (58.8%), and the majority of children had only two cardinal signs during flares. In adults, there was a higher interpersonal variability of the intercritical periods. Inflammatory markers measured during non-febrile periods were normal in children but altered in the totality of adults during febrile periods. A strong efficacy of corticosteroids in controlling the pediatric syndrome was observed, but response rates to steroids were less brilliant in adults. Colchicine and interleukin-1 inhibitors were used in the management of the steroid-resistant adult syndrome. Conversely, tonsillectomy was performed in a very low number of children, but was effective in 60.7% of adults when treated after 16 years. The mean age of disappearance of PFAPA symptoms has been 6.4 ± 2.4 years in children, while only 27% of adults have shown a complete drug-free symptom regression. CONCLUSIONS: A linear conformity of the PFAPA syndrome has been observed between pediatric and adult patients. PFAPA symptoms tended to disappear with no sequelae in 94.1% of children, while the disease was still active in almost 3/4 of adults at the time of our assessment.
INTRODUCTION: Analogies or differences of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in children and adults are barely known. The aim of our study was to compare the overall characteristics of a large cohort of patients, both children and adults, diagnosed with PFAPA syndrome. METHODS: In the last decade, we identified 120 children and 63 adults with periodically recurring fevers, who fulfilled the criteria for PFAPA diagnosis. The two subcohorts were analyzed according to demographic features, clinical manifestations, laboratory data, and responses to therapies. RESULTS: The mean age of onset was 2.4 ± 1.5 and 19.7 ± 10.3 years, respectively, in children and adults, while attacks occurred every 3.8 ± 0.8 and every 4.3 ± 2.3 weeks, respectively, in children and adults. A higher prevalence of exudative pharyngitis was observed in children (58.8%), and the majority of children had only two cardinal signs during flares. In adults, there was a higher interpersonal variability of the intercritical periods. Inflammatory markers measured during non-febrile periods were normal in children but altered in the totality of adults during febrile periods. A strong efficacy of corticosteroids in controlling the pediatric syndrome was observed, but response rates to steroids were less brilliant in adults. Colchicine and interleukin-1 inhibitors were used in the management of the steroid-resistant adult syndrome. Conversely, tonsillectomy was performed in a very low number of children, but was effective in 60.7% of adults when treated after 16 years. The mean age of disappearance of PFAPA symptoms has been 6.4 ± 2.4 years in children, while only 27% of adults have shown a complete drug-free symptom regression. CONCLUSIONS: A linear conformity of the PFAPA syndrome has been observed between pediatric and adult patients. PFAPA symptoms tended to disappear with no sequelae in 94.1% of children, while the disease was still active in almost 3/4 of adults at the time of our assessment.
Authors: S Padeh; N Brezniak; D Zemer; E Pras; A Livneh; P Langevitz; A Migdal; M Pras; J H Passwell Journal: J Pediatr Date: 1999-07 Impact factor: 4.406
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