周期性发热-阿弗他口炎-咽炎-淋巴结炎综合征的研究进展

Progress in the treatment of periodic fever-aphthous stomatitis-pharyngitis-lymphadenitis syndrome

周期性发热-阿弗他口炎-咽炎-淋巴结炎(PFAPA)综合征是儿童时期最常见的自身炎症性疾病,发病通常在5岁以前,但是近几年成人发病的报道也不断增多。该疾病的发病机制尚不明确,可能与多个炎症体相关基因突变导致白介素产生异常有关。PFAPA综合征主要表现为周期性的发热同时伴有口腔炎、咽炎及淋巴结炎至少其中一种症状,一部分患者还可见发热前驱症状,如疲倦、头痛、腹痛或易怒等。该疾病被认为是自限性疾病,所以临床上主要以对症治疗为主,治疗主要选择非甾体类抗炎药、皮质类固醇、西咪替丁、秋水仙碱及扁桃体切除术,近几年IL-1抑制剂也应用到治疗中,并且有不错的效果。现在PFAPA综合征还未修订出临床治疗指南,不同治疗方式的选择及长期治疗的治疗效果,仍需要进行更大样本量的随机临床对照实验。本文主要介绍有关PFAPA综合征的可用治疗方法。

Objective Periodic fever-aphthous stomatitis-pharyngitis and cervical adenitis(PFAPA) syndrome is the most common self-inflammatory disease in childhood, which usually occurs before the age of 5, but there have been increasing reports in adults in recent years. The pathogenesis of the disease is not clear yet, which may be related to the abnormal production of interleukin caused by multiple inflammatory body-related gene mutations. PFAPA is mainly characterized by periodic fever accompanied by stomatitis, pharyngitis and lymphadenitis, or at least one of the symptoms;fever prodromal symptoms such as fatigue, headache, abdominal pain or irritability can also been observed in some patients. The disease is considered to be a self-limited disease, clinical treatment is mainly symptomatic. It is mainly treated with non-steroidal anti-inflammatory drugs, corticosteroids, cimetidine, colchicine and tonsillectomy. In recent years, interleukin-1 inhibitors have also been used in the treatment, and have obtained a good effect. At present, the clinical treatment guidelines for PFAPA syndrome have not been revised. The choice of different treatment methods and the therapeutic effect of long-term treatment still need to carry out a larger sample size of randomized clinical controlled trials. This article mainly introduces the available treatments for PFAPA syndrome.