دورية أكاديمية
أعرض في EDS

Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation.

التفاصيل البيبلوغرافية
العنوان: Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation.
المؤلفون: Vyzhga Y; National Pirogov Memorial Medical University, Vinnytsya, Ukraine. yulia_tokarchuk@yahoo.com., Wittkowski H; Department of Pediatric Rheumatology and Immunology, University Hospital Munster, Munster, Germany., Hentgen V; Department for Pediatrics, National Referral Centre of Auto-Inflammatory Diseases and Inflammatory Amyloidosis, - CEREMAIA, Versailles Hospital, Le Chesnay (Paris), France., Georgin-Lavialle S; CEREMAIA (French Reference Center for Auto-Inflammatory Diseases and Inflammatory Amyloidosis), Kremlin-Bicêtre, France.; Department of Internal Medicine, Sorbonne University, Tenon Hospital (APHP), Paris, France., Theodoropoulou A; Department of Pediatrics, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland., Fuehner S; Department of Pediatric Rheumatology and Immunology, University Hospital Munster, Munster, Germany., Jesenak M; Department of Peadiatrics and Adolescent Medicine, Jesenius Faculty of Medicine in Martin, Comenius University, University Hospital Martin, Martin, Slovakia., Frenkel J; Department of Pediatric Immunology and Rheumatology, Wilhelmina Kinderziekenhuis, Utrecht, Netherlands., Papadopoulou-Alataki E; Department of Pediatrics, School of Medicine, Faculty of Health Sciences, Papageorgiou General Hospital, Fourth, Aristotle University of Thessaloniki, Thessaloniki, Greece., Anton J; Department of Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona. Institut de Recerca Sant Joan de Deu, Barcelona, Spain., Olivieri AN; Dipartimento Della Donna del Bambino E Di Chirurgia Generale E Specialistica, Università Degli Studi Della Campania L.Vanvitelli, Naples, Italy., Brunner J; Department of Pediatrics, Pediatric Rheumatology, Medical University Innsbruck, Innsbruck and Danube Private University Krems, Innsbruck, Austria., Sanchez J; Hospital Parc Taulí de Sabadell, Reumatologia Pediàtrica - Servei de Medicina Pediàtrica, Barcelona, Spain., Koné-Paut I; Department of Pediatric Rheumatology, National Referral Centre of Auto-Inflammatory Diseases and Inflammatory Amyloidosis, CEREMAIA, CHU de Biĉetre, APHP, University of Paris Sud, Le Kremlin Biĉetre, France., Fingerhutova S; Department of Paediatrics and Inherited Metabolic Disorders, Centre for Paediatric Rheumatology and Autoinflammatory Diseases 1st Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic., Pillet P; Pediatrics and Immunology, CHU Pellegrin, Bordeaux, France., Meinzer U; Department of General Paediatrics, Paediatric Infectious Disease and Internal Medicine, Assistance Publique-Hôpitaux de Paris, Robert Debré University Hospital, Université de Paris, Paris, France., Khubchandani R; Department of Pediatrics, Jaslok Hospital, Mumbai, India., Jansson A; Division of Pediatric Rheumatology and Immunology, Dr. Von Hauner Children's Hospital, University Hospital Munich, Munich, Germany., Haas JP; German Center for Paediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany., Berendes R; Marien Children's Hospital, Landshut, Germany., Kallinich T; German Rheumatism Research Center, Leibniz Institute Berlin Charité Universitätsmedizin Berlin, Paediatric Pneumology, Immunology and Critical Care Medicine and SPZ (Center for Chronically Sick Children), Berlin, Germany., Horneff G; Department of Pediatrics, Asklepios Clinic Sankt Augustin GmbH, Sankt Augustin, Germany., Lilienthal E; Department of Pediatrics, Ruhr University of Bochum, Bochum, Germany., Papa R; Centre for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genoa, Italy., Foell D; Department of Pediatric Rheumatology and Immunology, University Hospital Munster, Munster, Germany., Lainka E; Department of Pediatric Rheumatology, University Children's Hospital Essen, Essen, Germany., Caorsi R; Centre for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genoa, Italy., Gattorno M; Centre for Autoinflammatory Diseases and Immunodeficiencies, IRCCS Istituto Giannina Gaslini, Genoa, Italy., Hofer M; Department of Pediatrics, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland.
مؤلفون مشاركون: AID-Net JIRcohortEurofever/PRINTO
المصدر: Pediatric rheumatology online journal [Pediatr Rheumatol Online J] 2024 May 17; Vol. 22 (1), pp. 55. Date of Electronic Publication: 2024 May 17.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: BioMed Central Country of Publication: England NLM ID: 101248897 Publication Model: Electronic Cited Medium: Internet ISSN: 1546-0096 (Electronic) Linking ISSN: 15460096 NLM ISO Abbreviation: Pediatr Rheumatol Online J Subsets: MEDLINE
أسماء مطبوعة: Publication: 2007- : [London] : BioMed Central
Original Publication: [Chicago, Ill. : University of Chicago, 2003]-
مواضيع طبية MeSH: Registries* , Stomatitis, Aphthous*/diagnosis , Stomatitis, Aphthous*/epidemiology , Hereditary Autoinflammatory Diseases*/diagnosis , Lymphadenitis*/diagnosis , Lymphadenitis*/epidemiology , Pharyngitis*/diagnosis, Humans ; Child ; Europe/epidemiology ; Female ; Male ; Child, Preschool ; Adolescent ; Infant ; Retrospective Studies ; Fever/etiology ; Fever/diagnosis ; Recurrence
مستخلص: Background: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries.
Methods: Data of patients with PFAPA, SURF and uSAID were collected from 3 registries including detailed epidemiological, demographic and clinical data, results of the genetic testing and additional laboratory investigations with retrospective application of the modified Marshall and PRINTO/Eurofever classification criteria on the cohort of PFAPA patients and preliminary SURF criteria on uSAID/SURF patients.
Results: Clinical presentation of PFAPA is variable and some patients did not fit the conventional PFAPA criteria and exhibit different symptoms. Some patients did not meet the criteria for either PFAPA or SURF, highlighting the heterogeneity within these groups. The study also explored potential overlaps between PFAPA and SURF/uAID, revealing that some patients exhibited symptoms characteristic of both conditions, emphasizing the need for more precise classification criteria.
Conclusions: Patients with recurrent fevers without molecular diagnoses represent a clinically heterogeneous group. Improved classification criteria are needed for both PFAPA and SURF/uAID to accurately identify and manage these patients, ultimately improving clinical outcomes.
(© 2024. The Author(s).)
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فهرسة مساهمة: Keywords: AID-Net; Autoinflammatory diseases; Eurofever; JIR-cohort; PFAPA; SURF
تواريخ الأحداث: Date Created: 20240517 Date Completed: 20240518 Latest Revision: 20240520
رمز التحديث: 20240520
مُعرف محوري في PubMed: PMC11100049
DOI: 10.1186/s12969-024-00987-z
PMID: 38760816
قاعدة البيانات: MEDLINE
الوصف
تدمد:1546-0096
DOI:10.1186/s12969-024-00987-z