OBJECTIVES: This paper aims to describe clinical and laboratory features and disease outcome in a single-centre cohort of patients with PFAPA syndrome (Periodic Fever, Aphtous stomatitis, Pharyngitis, and Adenitis) and to test performance of diagnostic and therapeutic algorithms. METHODS: Patients fulfilling criteria were selected from the fever clinic population. Prospective follow-up together with recruitment of newly diagnosed patients followed pre-defined guidelines. Diagnostic and therapeutic algorithms and definitions of outcome and therapy response were formulated. Paired blood samples during febrile and afebrile periods were compared. RESULTS: Out of 176 patients referred for suspected periodic fever 125 children fulfilled criteria. Their age at onset was 23 months, median episode duration 3.5 days at 4-week intervals. Fever was associated with pharyngitis (91%), cervical adenitis (78%) and aphtae (41%). Among therapeutic options, episodic prednisone proved to be the most common first-line treatment. Administered to 77 patients, it reduced symptoms in 94%. Tonsillectomy led to the full symptom resolution in all 18 patients. Forty-six patients reached disease remission. CONCLUSIONS: Distribution of typical symptoms, response to therapies and disease outcome in a large patient cohort were documented. We offer diagnostic and therapeutic algorithms that have proven effective during this prospective trial. Our findings support the general belief of benign nature of this aetiologically unclear condition, despite proportion of patients having persistent disease for years. Maintenance of normal findings in afebrile intervals, striking response to a single dose of prednisone and normal growth and development together with spontaneous tendency towards prolongation of afebrile intervals are important confirmatory features of PFAPA syndrome.
OBJECTIVES: This paper aims to describe clinical and laboratory features and disease outcome in a single-centre cohort of patients with PFAPA syndrome (Periodic Fever, Aphtous stomatitis, Pharyngitis, and Adenitis) and to test performance of diagnostic and therapeutic algorithms. METHODS:Patients fulfilling criteria were selected from the fever clinic population. Prospective follow-up together with recruitment of newly diagnosed patients followed pre-defined guidelines. Diagnostic and therapeutic algorithms and definitions of outcome and therapy response were formulated. Paired blood samples during febrile and afebrile periods were compared. RESULTS: Out of 176 patients referred for suspected periodic fever 125 children fulfilled criteria. Their age at onset was 23 months, median episode duration 3.5 days at 4-week intervals. Fever was associated with pharyngitis (91%), cervical adenitis (78%) and aphtae (41%). Among therapeutic options, episodicprednisone proved to be the most common first-line treatment. Administered to 77 patients, it reduced symptoms in 94%. Tonsillectomy led to the full symptom resolution in all 18 patients. Forty-six patients reached disease remission. CONCLUSIONS: Distribution of typical symptoms, response to therapies and disease outcome in a large patient cohort were documented. We offer diagnostic and therapeutic algorithms that have proven effective during this prospective trial. Our findings support the general belief of benign nature of this aetiologically unclear condition, despite proportion of patients having persistent disease for years. Maintenance of normal findings in afebrile intervals, striking response to a single dose of prednisone and normal growth and development together with spontaneous tendency towards prolongation of afebrile intervals are important confirmatory features of PFAPA syndrome.
Authors: Kalpana Manthiram; Suzanne C Li; Jonathan S Hausmann; Gil Amarilyo; Karyl Barron; Hanna Kim; Simona Nativ; Geraldina Lionetti; Andrew Zeft; Donald Goldsmith; David Kimberlin; Kathryn Edwards; Fatma Dedeoglu; Sivia Lapidus Journal: Rheumatol Int Date: 2017-03-07 Impact factor: 2.631
Authors: Tatjana Welzel; Maren Ellinghaus; Anna L Wildermuth; Norbert Deschner; Susanne M Benseler; Jasmin B Kuemmerle-Deschner Journal: Front Pediatr Date: 2021-11-25 Impact factor: 3.418
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