Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome
ObjectiveTo 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).MethodsWe enrolled 74 adults diagnosed with PFAPA syndrome according to...
| Main Authors: | , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2017-08-01
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| Series: | Frontiers in Immunology |
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| Online Access: | http://journal.frontiersin.org/article/10.3389/fimmu.2017.01018/full |
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| author | Luca Cantarini Antonio Vitale Ludovico Luca Sicignano Giacomo Emmi Elena Verrecchia Isabella Patisso Lucia Cerrito Claudia Fabiani Gabriele Cevenini Bruno Frediani Mauro Galeazzi Donato Rigante Raffaele Manna |
| author_facet | Luca Cantarini Antonio Vitale Ludovico Luca Sicignano Giacomo Emmi Elena Verrecchia Isabella Patisso Lucia Cerrito Claudia Fabiani Gabriele Cevenini Bruno Frediani Mauro Galeazzi Donato Rigante Raffaele Manna |
| author_sort | Luca Cantarini |
| collection | DOAJ |
| description | ObjectiveTo 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).MethodsWe 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.ResultsAccording to the multivariate analysis, the clinical variables that discriminated PFAPA patients were: fever episodes associated with cervical lymphadenitis (OR = 92; p < 0.0001), fever attacks associated with erythematous pharyngitis (OR = 231; p < 0.0001), increased inflammatory markers during fever attacks (OR = 588; p = 0.001), and the lack of clinical and laboratory signs of inflammation between flares (OR = 1202; p < 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.Conclusionwe 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. |
| first_indexed | 2024-12-10T16:42:32Z |
| format | Article |
| id | doaj.art-edf40a503e494aeea062ff9fe5ad83fe |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-12-10T16:42:32Z |
| publishDate | 2017-08-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Immunology |
| spelling | doaj.art-edf40a503e494aeea062ff9fe5ad83fe2022-12-22T01:41:10ZengFrontiers Media S.A.Frontiers in Immunology1664-32242017-08-01810.3389/fimmu.2017.01018289174Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) SyndromeLuca Cantarini0Antonio Vitale1Ludovico Luca Sicignano2Giacomo Emmi3Elena Verrecchia4Isabella Patisso5Lucia Cerrito6Claudia Fabiani7Gabriele Cevenini8Bruno Frediani9Mauro Galeazzi10Donato Rigante11Raffaele Manna12Research 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, ItalyResearch 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, ItalyPeriodic Fevers Research Center, Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, ItalyDepartment of Experimental and Clinical Medicine, University of Florence, Florence, ItalyPeriodic Fevers Research Center, Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, ItalyPeriodic Fevers Research Center, Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, ItalyPeriodic Fevers Research Center, Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, ItalyDepartment of Ophthalmology, Humanitas Research Center, Rozzano, ItalyDepartment of Biotechnology, Chemistry and Pharmacy, University of Siena, Siena, ItalyResearch 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, ItalyResearch 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, ItalyInstitute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, ItalyPeriodic Fevers Research Center, Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, ItalyObjectiveTo 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).MethodsWe 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.ResultsAccording to the multivariate analysis, the clinical variables that discriminated PFAPA patients were: fever episodes associated with cervical lymphadenitis (OR = 92; p < 0.0001), fever attacks associated with erythematous pharyngitis (OR = 231; p < 0.0001), increased inflammatory markers during fever attacks (OR = 588; p = 0.001), and the lack of clinical and laboratory signs of inflammation between flares (OR = 1202; p < 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.Conclusionwe 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.http://journal.frontiersin.org/article/10.3389/fimmu.2017.01018/fullPFAPA syndromeautoinflammatory diseasedifferential diagnosisdiagnostic criteriaadultsfever of unknown origin |
| spellingShingle | Luca Cantarini Antonio Vitale Ludovico Luca Sicignano Giacomo Emmi Elena Verrecchia Isabella Patisso Lucia Cerrito Claudia Fabiani Gabriele Cevenini Bruno Frediani Mauro Galeazzi Donato Rigante Raffaele Manna Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Frontiers in Immunology PFAPA syndrome autoinflammatory disease differential diagnosis diagnostic criteria adults fever of unknown origin |
| title | Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome |
| title_full | Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome |
| title_fullStr | Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome |
| title_full_unstemmed | Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome |
| title_short | Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome |
| title_sort | diagnostic criteria for adult onset periodic fever aphthous stomatitis pharyngitis and cervical adenitis pfapa syndrome |
| topic | PFAPA syndrome autoinflammatory disease differential diagnosis diagnostic criteria adults fever of unknown origin |
| url | http://journal.frontiersin.org/article/10.3389/fimmu.2017.01018/full |
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