Rigante Donato, Candelli Marcello
Department of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Rome, Italy.
Periodic Fever Research Center, Università Cattolica Sacro Cuore, 00168 Rome, Italy.
Children (Basel). 2024 Dec 23;11(12):1559. doi: 10.3390/children11121559.
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome, often referred to as PFAPA syndrome, may enigmatically recur for an undetermined time in affected children: a potential reason to explain its recurring pattern for an unpredictable period or its self-limitation is currently unknown. We explored the relationship between different general, demographic, clinical, and laboratory features of PFAPA children and disease evolution over the course of a decade. We have retrospectively screened 150 Italian children with a history of PFAPA syndrome attending the Outpatients Clinic of Pediatric Rheumatology in our Institution during the period 2014-2024, all without any recognized chronic diseases: 88 males, 62 females, mean age at onset of 2.5 ± 1.7 years, age range of 0.3-9.4 years, and mean age at diagnosis of 4.5 ± 2.0 years. The whole cohort of PFAPA patients had been followed up for a median period of 5 years (IQR: 4-7). After dividing patients into two groups based on either the disappearance or persistence of PFAPA symptoms during follow-up, we found that positive family history of recurring fevers, cervical lymphadenopathy, arthralgia, myalgia, and breastfeeding for more than 6 months were associated with the disappearance of febrile attacks for at least six months. Performing a multivariate analysis adjusted for sex and age, we found that only breastfeeding duration longer than 6 months and higher education level of PFAPA patients' mothers were independently associated with the resolution of PFAPA symptoms.
周期性发热、口疮性口炎、咽炎和颈淋巴结炎综合征,通常被称为PFAPA综合征,可能会在患病儿童中莫名其妙地反复出现一段不确定的时间:目前尚不清楚其在不可预测的时间段内反复出现的模式或自我限制的潜在原因。我们探讨了PFAPA患儿不同的一般、人口统计学、临床和实验室特征与十年病程中疾病演变之间的关系。我们回顾性筛查了2014年至2024年期间在我们机构儿科风湿病门诊就诊的150名有PFAPA综合征病史的意大利儿童,他们均无任何公认的慢性疾病:88名男性,62名女性,发病时平均年龄为2.5±1.7岁,年龄范围为0.3至9.4岁,诊断时平均年龄为4.5±2.0岁。整个PFAPA患者队列的中位随访期为5年(四分位间距:4至7年)。在根据随访期间PFAPA症状的消失或持续将患者分为两组后,我们发现反复发热的阳性家族史、颈淋巴结病、关节痛、肌痛以及母乳喂养超过6个月与发热发作消失至少6个月相关。在对性别和年龄进行校正的多变量分析中,我们发现只有母乳喂养持续时间超过6个月以及PFAPA患者母亲的教育水平较高与PFAPA症状的缓解独立相关。