Cantarini Luca, Vitale Antonio, Sicignano Ludovico Luca, Emmi Giacomo, Verrecchia Elena, Patisso Isabella, Cerrito Lucia, Fabiani Claudia, Cevenini Gabriele, Frediani Bruno, Galeazzi Mauro, Rigante Donato, Manna Raffaele
Research Center of Systemic Autoinflammatory Diseases, Behçet's Disease and Rheumatology-Ophthalmology Collaborative Uveitis Center, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy.
Periodic Fevers Research Center, Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy.
Front Immunol. 2017 Aug 24;8:1018. doi: 10.3389/fimmu.2017.01018. eCollection 2017.
To identify a set of variables that could discriminate patients with adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome from subjects with fever of unknown origin (FUO).
We enrolled 74 adults diagnosed with PFAPA syndrome according to the currently used pediatric diagnostic criteria and 62 additional patients with FUO. After having collected clinical and laboratory data from both groups, univariate and multivariate analyses were performed to identify the variables associated with PFAPA diagnosis. Odds ratio (OR) values, their statistical significance, and corresponding 95% confidence interval (CI) were evaluated for each diagnostic factor both at the univariate and multivariate analyses. Diagnostic accuracy was evaluated by the area under receiver operating characteristic (ROC) curve, while the leave-one-out cross-validation procedure was used to ensure that the model maintains the same diagnostic power when applied to new data.
According to the multivariate analysis, the clinical variables that discriminated PFAPA patients were: fever episodes associated with cervical lymphadenitis (OR = 92; < 0.0001), fever attacks associated with erythematous pharyngitis (OR = 231; < 0.0001), increased inflammatory markers during fever attacks (OR = 588; = 0.001), and the lack of clinical and laboratory signs of inflammation between flares (OR = 1202; < 0.0001). These variables were considered for a diagnostic model which accounted for their OR values. The diagnostic accuracy of the proposed set of criteria corresponded to an area under ROC curve of 0.978 (95% CI 0.958-0.998), with a model sensitivity and specificity equal to 93.4% (95% CI 87.5-96.5%) and 91.7% (95% CI 82.8-96.7%), respectively.
we have provided herein a set of clinical diagnostic criteria for adult-onset PFAPA syndrome. Our criteria represent an easy-to-use diagnostic tool aimed at identifying PFAPA patients among subjects with FUO with a high-predictive potential, as shown by its very high sensitivity and specificity.
确定一组能够区分成人发作性周期性发热、口疮性口炎、咽炎和颈淋巴结炎（PFAPA）综合征患者与不明原因发热（FUO）患者的变量。
我们纳入了74名根据目前使用的儿科诊断标准被诊断为PFAPA综合征的成年人以及另外62名FUO患者。在收集了两组的临床和实验室数据后，进行单变量和多变量分析以确定与PFAPA诊断相关的变量。在单变量和多变量分析中，对每个诊断因素评估比值比（OR）值、其统计学意义以及相应的95%置信区间（CI）。通过受试者操作特征（ROC）曲线下面积评估诊断准确性，同时采用留一法交叉验证程序以确保该模型在应用于新数据时保持相同的诊断能力。
根据多变量分析，区分PFAPA患者的临床变量为：与颈淋巴结炎相关的发热发作（OR = 92；< 0.0001）、与红斑性咽炎相关的发热发作（OR = 231；< 0.0001）、发热发作期间炎症标志物升高（OR = 588；= 0.001）以及发作间期缺乏临床和实验室炎症体征（OR = 1202；< 0.0001）。考虑这些变量构建了一个诊断模型，该模型考虑了它们的OR值。所提出的这组标准的诊断准确性对应于ROC曲线下面积为0.978（95% CI 0.958 - 0.998），模型敏感性和特异性分别为93.4%（95% CI 87.5 - 96.5%）和91.7%（95% CI 82.8 - 96.7%）。
我们在此提供了一组成人发作性PFAPA综合征的临床诊断标准。我们的标准是一种易于使用的诊断工具，旨在从FUO患者中识别PFAPA患者，其具有很高的预测潜力，这体现在其非常高的敏感性和特异性上。
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