OBJECTIVE: The purpose of this study is to share our experience about clinical findings, natural course, and treatment response rates of a large cohort of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. MATERIALS AND METHODS: Medical records of patients who were diagnosed with PFAPA syndrome between January 2010 and May 2021 at Istanbul University-Cerrahpasa Cerrahpasa Medical Faculty pediatric rheumatology department were reviewed retrospectively. RESULTS: A total of 607 patients (females: 277, males: 330) with PFAPA syndrome were included. The median duration of episodes was 3 (1-15; interquartile range (IQR) 3-5) days, and the median interval between episodes was 20 days (5-120; IQR 15-30). The median age at the last attack and median disease duration were 66 (24-168; IQR 48-84) months and 40 (4-132; IQR 27.5-60) months, respectively. Fever (100%) was the most common clinical finding, followed by pharyngitis/exudative tonsillitis in 594 (97.9%), aphthous stomatitis in 308 (50.7%), cervical lymphadenopathy in 278 (45.8%), abdominal pain in 249 (41%), and arthralgia in 228 (37.6%) of the patients. Among the clinical findings, there was no statistical difference according to gender, except for cervical lymphadenitis being higher in males (P < .001). Of the patients who were given steroids during attacks, 94.6% were responsive. Colchicine was effective in 93 (63.7%) patients. The disease episodes ceased in 313 (95.4%) of patients who had tonsillectomy/adenoidectomy. CONCLUSIONS: Clinicians should be alert for additional symptoms such as abdominal pain, arthralgia, and headache apart from the cardinal signs. Although tonsillectomy is highly effective, its use is controversial. Colchicine may be a good alternative for prophylaxis.
OBJECTIVE: The purpose of this study is to share our experience about clinical findings, natural course, and treatment response rates of a large cohort of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. MATERIALS AND METHODS: Medical records of patients who were diagnosed with PFAPA syndrome between January 2010 and May 2021 at Istanbul University-Cerrahpasa Cerrahpasa Medical Faculty pediatric rheumatology department were reviewed retrospectively. RESULTS: A total of 607 patients (females: 277, males: 330) with PFAPA syndrome were included. The median duration of episodes was 3 (1-15; interquartile range (IQR) 3-5) days, and the median interval between episodes was 20 days (5-120; IQR 15-30). The median age at the last attack and median disease duration were 66 (24-168; IQR 48-84) months and 40 (4-132; IQR 27.5-60) months, respectively. Fever (100%) was the most common clinical finding, followed by pharyngitis/exudative tonsillitis in 594 (97.9%), aphthous stomatitis in 308 (50.7%), cervical lymphadenopathy in 278 (45.8%), abdominal pain in 249 (41%), and arthralgia in 228 (37.6%) of the patients. Among the clinical findings, there was no statistical difference according to gender, except for cervical lymphadenitis being higher in males (P < .001). Of the patients who were given steroids during attacks, 94.6% were responsive. Colchicine was effective in 93 (63.7%) patients. The disease episodes ceased in 313 (95.4%) of patients who had tonsillectomy/adenoidectomy. CONCLUSIONS: Clinicians should be alert for additional symptoms such as abdominal pain, arthralgia, and headache apart from the cardinal signs. Although tonsillectomy is highly effective, its use is controversial. Colchicine may be a good alternative for prophylaxis.
Authors: Ezgi D Batu; Fehime Kara Eroğlu; Paul Tsoukas; Jonathan S Hausmann; Yelda Bilginer; Margaret A Kenna; Greg R Licameli; Robert C Fuhlbrigge; Seza Özen; Fatma Dedeoğlu Journal: Arthritis Care Res (Hoboken) Date: 2016-12 Impact factor: 4.794
Authors: E Deniz Gozen; Mehmet Yildiz; Sinem Kara; Firat Tevetoglu; Fatih Haslak; Amra Adrovic; Sezgin Sahin; Kenan Barut; İpek Ulkersoy; Neslihan Gücüyener; Aybuke Gunalp; H Murat Yener; Mehmet Ada; Ozgur Kasapcopur Journal: Rheumatol Int Date: 2022-09-18 Impact factor: 3.580
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