中华临床免疫和变态反应杂志2017，Vol.11Issue(4)：339-343,5.DOI:10.3969/j.issn.1673-8705.2017.04.006
成人周期性发热-阿弗它口炎-咽炎-淋巴结炎综合征
Clinical Features of Adult Patients with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis
摘要
Abstract
Objective Periodic fever, aphthous stomatitis, pharyngitis, and adenits ( PFAPA) syndrome is a multifactorial autoinflammatory disease ( AUID) with unknown etiology .We aimed to inves-tigate the clinical features , treatment, and prognosis of Chinese adult patients with PFAPA syndrome . Methods We evaluated the clinical data of 52 adult patients (≥14 years old ) with recurrent fever of unknown origin who were suspected monogenic AUIDs in the period through April 2015 to April 2017, at the adult AUIDs center , Department of Rheumatology , Peking Union Medical College Hospital .Gene tests were examined to exclude monogenic AUIDs .The clinical features were evaluated for patients with PFAPA syndrome, and compared with those of adult PFAPA patients from centers abroad .Results A total of 7 (13.5％) adult patients were diagnosed as PFAPA syndrome , including 6 (85.7％) men.The mean age of disease onset was (27±10) years.Periodic fever was the main character of PFAPA syndrome , with a median attack time of 3.5 days, and median interval of 4 weeks.Pharyngitis, adenitis and aphthous sto-matitis were present in 85.7％, 57.1％and 42.9％, respectively .Corticosteroids used during attack were effective in 3 patients.Conclusion PFAPA syndrome should be considered in every patient of whatever age suffering from recurrent fevers of unknown origin , especially those with pharyngitis , cervical adenopa-thy and aphthous stomatitis .关键词
自身炎症性疾病/成人起病/不明原因发热/周期性发热-阿弗它口炎-咽炎-淋巴结炎综合征Key words
autoinflammatory diseases/adult-onset/fever of unknown origin/PFAPA syndrome分类
医药卫生引用本文复制引用
赵建红,吴迪,沈敏..成人周期性发热-阿弗它口炎-咽炎-淋巴结炎综合征[J].中华临床免疫和变态反应杂志,2017,11(4):339-343,5.基金项目
国家自然科学基金(81501405),中国医学科学院医学与健康科技创新工程项目(2017-I2M-3-001),北京协和医学院协和青年基金(中央高校基本科研业务费专项资金)(3332015092) （81501405）
