Kyvsgaard Nini, Mikkelsen Torben, Korsholm Jakob, Veirum Jens Erik, Herlin Troels
Department of Paediatrics, Aarhus University Hospital, Skejby, Aarhus N, Denmark.
Dan Med J. 2012 Jul;59(7):A4452.
The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a non-hereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. PFAPA does not cause long-lasting sequelae. An early diagnosis provides treatment possibilities for the patient and comfort to the family.
This study is a retrospective review of the medical records of patients diagnosed with PFAPA and admitted to our clinic from January 1999 to January 2010 (n = 31).
The study population (n = 31) consisted of 21 males and ten females: 30 Caucasians and 1 Asian. Normal growth was seen in 30 patients. The median age at onset was 33 months. The mean duration of fever episodes was 4.45 days (95% confidence interval (CI): 3.92-4.98 days), and the mean duration of intervals between fever episodes was 29.66 days (95% CI: 25.31-34.01 days). Concomitantly with the fever, all patients had characteristic symptoms. All patients were asymptomatic in between their fever episodes. Prodromal symptoms were seen in 12 patients. Oral prednisolone was used in 24 patients and caused immediate fever reduction in 87.5%. A reduction in the duration of the asymptomatic interval after treatment was seen in 75.0%. Tonsillectomy was performed in 20 of the 31 patients causing cessation of fever episodes in 70%. Fever episodes continued in 15%, and the postoperative status remained unknown in the last 15%. Spontaneous resolution was seen in four patients. The diagnostic delay had a median duration of 28 months (range 2-160 months).
The long diagnostic delay of PFAPA gives cause for concern and it indicates a need for greater awareness of the disease so that the diagnosis may be made earlier.
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周期性发热、阿弗他口炎、咽炎和颈淋巴结炎（PFAPA）综合征是一种属于自身炎症性疾病的非遗传性特发性发热综合征。PFAPA不会导致长期后遗症。早期诊断为患者提供了治疗可能性，并让家人安心。
本研究是对1999年1月至2010年1月在我院确诊并入院的PFAPA患者的病历进行回顾性分析（n = 31）。
研究对象（n = 31）包括21名男性和10名女性：30名白种人和1名亚洲人。30名患者生长发育正常。发病的中位年龄为33个月。发热发作的平均持续时间为4.45天（95%置信区间（CI）：3.92 - 4.98天），发热发作间期的平均持续时间为29.66天（95% CI：25.31 - 34.01天）。发热时，所有患者均有特征性症状。发热发作间期所有患者均无症状。12名患者出现前驱症状。24名患者使用口服泼尼松龙，87.5%的患者发热立即缓解。75.0%的患者治疗后无症状间期缩短。31名患者中有20名接受了扁桃体切除术，70%的患者发热发作停止。15%的患者发热发作仍持续，最后15%的患者术后情况不明。4名患者自行缓解。诊断延迟的中位持续时间为28个月（范围2 - 160个月）。
PFAPA的诊断延迟时间长令人担忧，这表明需要提高对该疾病的认识，以便能更早地做出诊断。
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