BACKGROUND: The pathophysiology of PFAPA syndrome, mainly characterised by regularly recurring periodic fevers associated with aphthous stomatitis, pharyngitis and/or lymphadenitis, and mostly occurring in the paediatric setting, resembles an acquired autoinflammatory disease. The description of PFAPA syndrome in adult patients is largely increasing. OBJECTIVES: To recognise PFAPA syndrome in a group of adult patients evaluated for recurrent fevers in our Rheumatology Unit. METHODS: To apply current diagnostic criteria for PFAPA syndrome in a group of 359 adults with unexplained recurrent fevers monitored in our Unit between January 2007 and June 2011. RESULTS: We have found 17 out of 359 patients fulfilling the diagnosis of PFAPA syndrome: these patients (10 males, 7 females) were Caucasian with a mean age of 33.3±9.5 years, had recurrent febrile episodes begun at a mean age of 25.9±8.3 years and a mean number of episodes of 8.3±5.2 per year with a mean duration of 5.5±1.8 days. In particular, 7/17 patients had the 3 cardinal signs, the other 10 had a combination of 2 signs. Corticosteroids were given in 14/17 patients; tonsillectomy was performed in 9/17 patients: corticosteroid responsiveness and tonsillectomy efficacy were observed respectively in 11 and 2 patients. CONCLUSIONS: Our case highlights the importance of considering PFAPA syndrome in adults presenting with unexplained recurrent fevers and symptoms commonly encountered in general medical practice.
BACKGROUND: The pathophysiology of PFAPA syndrome, mainly characterised by regularly recurring periodic fevers associated with aphthous stomatitis, pharyngitis and/or lymphadenitis, and mostly occurring in the paediatric setting, resembles an acquired autoinflammatory disease. The description of PFAPA syndrome in adult patients is largely increasing. OBJECTIVES: To recognise PFAPA syndrome in a group of adult patients evaluated for recurrent fevers in our Rheumatology Unit. METHODS: To apply current diagnostic criteria for PFAPA syndrome in a group of 359 adults with unexplained recurrent fevers monitored in our Unit between January 2007 and June 2011. RESULTS: We have found 17 out of 359 patients fulfilling the diagnosis of PFAPA syndrome: these patients (10 males, 7 females) were Caucasian with a mean age of 33.3±9.5 years, had recurrent febrile episodes begun at a mean age of 25.9±8.3 years and a mean number of episodes of 8.3±5.2 per year with a mean duration of 5.5±1.8 days. In particular, 7/17 patients had the 3 cardinal signs, the other 10 had a combination of 2 signs. Corticosteroids were given in 14/17 patients; tonsillectomy was performed in 9/17 patients: corticosteroid responsiveness and tonsillectomy efficacy were observed respectively in 11 and 2 patients. CONCLUSIONS: Our case highlights the importance of considering PFAPA syndrome in adults presenting with unexplained recurrent fevers and symptoms commonly encountered in general medical practice.
Authors: Luca Cantarini; Antonio Vitale; Giulia Bersani; Laura Martin Nieves; Marco Cattalini; Giuseppe Lopalco; Francesco Caso; Luisa Costa; Florenzo Iannone; Giovanni Lapadula; Mauro Galeazzi; Angela Ceribelli; Enrico Brunetta; Carlo Selmi; Donato Rigante Journal: Clin Rheumatol Date: 2015-02-10 Impact factor: 2.980
Authors: Cristian Quintana-Ortega; Elena Seoane-Reula; Laura Fernández; Marisol Camacho; Peter Olbrich; Olaf Neth; Sara Murias; Clara Udaondo; Agustín Remesal; Cristina Calvo; Rosa Alcobendas Journal: Eur J Rheumatol Date: 2020-09-18
Authors: Donato Rigante; Antonio Vitale; Marco Francesco Natale; Giuseppe Lopalco; Laura Andreozzi; Bruno Frediani; Francesca D'Errico; Florenzo Iannone; Luca Cantarini Journal: Clin Rheumatol Date: 2016-06-01 Impact factor: 2.980
Authors: Marco Cattalini; Martina Soliani; Giuseppe Lopalco; Donato Rigante; Luca Cantarini Journal: Intern Emerg Med Date: 2016-05-25 Impact factor: 3.397