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Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation
Y. Vyzhga, H. Wittkowski, V. Hentgen, S. Georgin-Lavialle, A. Theodoropoulou, S. Fuehner, M. Jesenak, J. Frenkel, E. Papadopoulou-Alataki, J. Anton, AN. Olivieri, J. Brunner, J. Sanchez, I. Koné-Paut, S. Fingerhutova, P. Pillet, U. Meinzer, R....
Language English Country England, Great Britain
Document type Journal Article
NLK
BioMedCentral
from 2007-01-12
BioMedCentral Open Access
from 2007
Directory of Open Access Journals
from 2007
Free Medical Journals
from 2007
PubMed Central
from 2007
Europe PubMed Central
from 2007
ProQuest Central
from 2009-01-01
Open Access Digital Library
from 2007-01-01
Open Access Digital Library
from 2007-01-01
Health & Medicine (ProQuest)
from 2009-01-01
ROAD: Directory of Open Access Scholarly Resources
from 2003
Springer Nature OA/Free Journals
from 2007-12-01
- MeSH
- Stomatitis, Aphthous * diagnosis epidemiology MeSH
- Hereditary Autoinflammatory Diseases * diagnosis MeSH
- Child MeSH
- Pharyngitis * diagnosis MeSH
- Fever etiology diagnosis MeSH
- Infant MeSH
- Humans MeSH
- Lymphadenitis * diagnosis epidemiology MeSH
- Adolescent MeSH
- Child, Preschool MeSH
- Recurrence MeSH
- Registries * MeSH
- Retrospective Studies MeSH
- Check Tag
- Child MeSH
- Infant MeSH
- Humans MeSH
- Adolescent MeSH
- Male MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- Europe MeSH
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.
CEREMAIA Kremlin Bicêtre France
Department of Internal Medicine Sorbonne University Tenon Hospital Paris France
Department of Pediatric Immunology and Rheumatology Wilhelmina Kinderziekenhuis Utrecht Netherlands
Department of Pediatric Rheumatology and Immunology University Hospital Munster Munster Germany
Department of Pediatric Rheumatology University Children's Hospital Essen Essen Germany
Department of Pediatrics Asklepios Clinic Sankt Augustin GmbH Sankt Augustin Germany
Department of Pediatrics Centre Hospitalier Universitaire Vaudois Lausanne Switzerland
Department of Pediatrics Jaslok Hospital Mumbai India
Department of Pediatrics Ruhr University of Bochum Bochum Germany
German Center for Paediatric and Adolescent Rheumatology Garmisch Partenkirchen Germany
Marien Children's Hospital Landshut Germany
National Pirogov Memorial Medical University Vinnytsya Ukraine
References provided by Crossref.org
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- $a 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.
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