Abstract:
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in pediatric patients. It is clinically characterized by fever flares lasting 3-7 days, reappearing every 2-8 weeks with a distinctive clockwork regularity. PFAPA generally begins before 5 years of age and usually ceases 3-5 years after onset. Recurrences may be observed in adolescence and adulthood in up to 20% of cases. The authors aim to describe a case of PFAPA recurrence in adolescence temporally associated with allergen-specific immunotherapy (ASIT). A 16-year-old female patient was referred to the rheumatology unit due to recurrent episodes of fever one month after initiating ASIT for allergic rhinitis. These episodes occurred every 4 weeks and lasted 3 days. During these episodes, she also presented with a sore throat, tonsillar exudates, and cervical lymphadenopathy. Abortive treatment with oral prednisolone was attempted in these episodes, with complete resolution of fever after a single dose. After reviewing her medical background, she had previously experienced febrile episodes accompanied by aphthous ulcers and tonsillar exudates occurring every 7-8 weeks from age 2-7. The etiopathogenesis of PFAPA remains uncertain. Environmental triggers, particularly those with immunomodulator effects, may interfere with the immune responses responsible for PFAPA occurrence, but the mechanisms are still unclear. The authors describe the first report of the reappearance of PFAPA flares, possibly due to ASIT. Further studies are needed to fully clarify if ASIT constitutes a true environmental trigger of PFAPA.
摘要:
周期性发烧,口疮性口炎,咽炎,和颈腺炎(PFAPA)综合征是儿科患者最常见的周期性发热综合征。它的临床特征是持续3-7天的发烧耀斑,每2-8周重现一次,具有独特的发条规律。PFAPA通常在5岁之前开始,通常在发病后3-5年停止。在多达20%的病例中,青春期和成年期可能会出现复发。作者旨在描述一例与过敏原特异性免疫疗法(ASIT)相关的青春期PFAPA复发。一名16岁的女性患者因过敏性鼻炎开始ASIT后一个月反复发烧而被转诊到风湿病科。这些发作每4周发生一次,持续3天。在这些情节中,她还出现了喉咙痛,扁桃体分泌物,和颈淋巴结病。在这些发作中尝试口服泼尼松龙进行流产治疗,单次剂量后发烧完全消退。在回顾了她的医学背景后,从2~7岁开始,她曾经历过发热发作,伴有口疮性溃疡和扁桃体渗出,每7~8周发生一次.PFAPA的病因仍不确定。环境触发因素,特别是那些有免疫调节剂作用的,可能会干扰导致PFAPA发生的免疫反应,但机制尚不清楚。作者描述了PFAPA耀斑重现的第一份报告,可能是由于ASIT。需要进一步的研究来充分澄清ASIT是否构成PFAPA的真正环境触发因素。