Rigante Donato, Gentileschi Stefano, Vitale Antonio, Tarantino Giusyda, Cantarini Luca
Institute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico A. Gemelli, Rome, Italy.
Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases Behçet's Disease Clinic, University of Siena, Siena, Italy.
Isr Med Assoc J. 2017 Jul;19(7):444-447.
Fevers recurring at a nearly predictable rate every 3-8 weeks are the signature symptom of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome, an acquired autoinflammatory disorder which recurs in association with at least one sign among aphthous stomatitis, pharyngitis, and/or cervical lymph node enlargement without clinical signs related to upper respiratory airways or other localized infections. The disease usually has a rather benign course, although it might relapse during adulthood after a spontaneous or treatment-induced resolution in childhood. The number of treatment choices currently available for PFAPA syndrome has grown in recent years, but data from clinical trials dedicated to this disorder are limited to small cohorts of patients or single case reports. The response of PFAPA patients to a single dose of corticosteroids is usually striking, while little data exist for treatment with cimetidine and colchicine. Preliminary interesting results have been published with regard to vitamin D supplementation in PFAPA syndrome, while inhibition of interleukin-1 might represent an intriguing treatment for PFAPA patients who have not responded to standard therapies. Tonsillectomy has been proven curative in many studies related to PFAPA syndrome, although the evidence of its efficacy is not widely shared by different specialists, including pediatricians, rheumatologists and otorhynolaryngologists.
每3 - 8周以近乎可预测的频率反复发热是周期性发热、口疮性口炎、咽炎、颈淋巴结炎（PFAPA）综合征的标志性症状，这是一种后天性自身炎症性疾病，复发时伴有口疮性口炎、咽炎和/或颈淋巴结肿大中的至少一种体征，且无与上呼吸道或其他局部感染相关的临床体征。该疾病通常病程较为良性，尽管在儿童期自发缓解或经治疗缓解后，成年期可能会复发。近年来，目前可用于PFAPA综合征的治疗选择有所增加，但针对该疾病的临床试验数据仅限于小部分患者队列或单病例报告。PFAPA患者对单剂量皮质类固醇的反应通常很显著，而关于西咪替丁和秋水仙碱治疗的数据很少。关于PFAPA综合征补充维生素D已发表了初步有趣的结果，而抑制白细胞介素 - 1可能是对标准疗法无反应的PFAPA患者的一种有吸引力的治疗方法。在许多与PFAPA综合征相关的研究中，扁桃体切除术已被证明具有治愈效果，尽管包括儿科医生、风湿病学家和耳鼻喉科医生在内的不同专家对其疗效证据的认可度并不高。
