Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by recurrent fever flares accompanied by symptoms and signs forming the disease acronym and alternating with asymptomatic periods. Despite the disease having a generally favorable outcome, with spontaneous remission after a few years, it does have a major impact on the quality of life of the child and his or her relatives. Beside symptomatic medications during fever flares, the most used treatment consists of a single dose of corticosteroids at flare onset to interrupt the attack; fever resolves usually within a few hours, but often with a shorter interval between the attacks. For these patients, colchicine has been shown to decrease the frequency of the flares. Other medications were also reported in case series of patients with PFAPA syndrome. These include the interleukin-1 blocker anakinra to treat flares, cimetidine (which showed no convincing effect), and other drugs with anecdotal use. The pediatrician faces a difficult question: should they wait for spontaneous disease resolution and only treat the flares medically, or should they propose tonsillectomy that may induce remission? Due to the lack of strong evidence, the answer will vary on an individual basis, depending on the impact on the patient’s quality of life. The choice of the best therapeutic strategy will be based on the response to the flare-based therapy and on disease severity (quality of life, school performances); long-term data on the remission rate under the different therapeutic strategies are currently missing. More randomized controlled studies are needed to help the treating physician to choose the best therapy, as well as real-life long-term data to evaluate the long-term outcome of children with PFAPA syndrome.

中文翻译：

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为什么以及如何治疗周期性发热、阿弗他口炎、咽炎和宫颈腺炎 (PFAPA) 综合征？

周期性发热、口疮性口炎、咽炎和宫颈腺炎 (PFAPA) 综合征的特征是反复发热，伴有形成疾病首字母缩略词的症状和体征，并与无症状期交替出现。尽管该疾病具有普遍良好的结果，几年后可自发缓解，但它确实对儿童及其亲属的生活质量产生重大影响。除了在发热发作期间使用对症药物外，最常用的治疗方法是在发热发作时使用单剂量的皮质类固醇来中断发作；发烧通常会在几小时内消退，但发作之间的间隔通常较短。对于这些患者，秋水仙碱已被证明可以减少发作的频率。在 PFAPA 综合征患者的病例系列中还报告了其他药物。这些包括用于治疗耀斑的白介素 1 阻滞剂阿那白滞素、西咪替丁（没有显示出令人信服的效果）和其他有轶事用途的药物。儿科医生面临着一个难题：他们应该等待疾病自发消退并仅对耀斑进行药物治疗，还是应该建议可能导致缓解的扁桃体切除术？由于缺乏强有力的证据，答案会因人而异，取决于对患者生活质量的影响。最佳治疗策略的选择将基于对突发治疗的反应和疾病严重程度（生活质量、学校表现）；目前缺乏不同治疗策略下缓解率的长期数据。