Is the performance of the international severity scoring system for familial mediterranean fever in children better than other scoring systems?
MEFV mutations
children
disease severity score
familial mediterranean fever
international severity scoring system for familial mediterranean fever
Journal
International journal of clinical practice
ISSN: 1742-1241
Titre abrégé: Int J Clin Pract
Pays: India
ID NLM: 9712381
Informations de publication
Date de publication:
Nov 2021
Nov 2021
Historique:
revised:
03
07
2021
received:
14
04
2021
accepted:
21
07
2021
pubmed:
31
7
2021
medline:
25
2
2023
entrez:
30
7
2021
Statut:
ppublish
Résumé
Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterised by recurrent episodes of fever and serosal inflammation with elevated acute phase reactants. Assessing the severity of the disease may be useful in identifying colchicine-resistant patients. The aim of this study is to determine the disease severity of FMF patients according to the Pras, Mor, and International Severity Scoring System for Familial Mediterranean Fever (ISSF) scoring systems and to evaluate the consistency of these three systems. The medical records of patients with FMF were retrospectively reviewed. Demographic features, family history of FMF, clinical characteristics at disease onset, laboratory features, Mediterranean fever genetic mutations, treatment regimens, and disease courses were recorded. A total of 205 patients (116 girls) were included in the study. The mean age of the patients was 13.3 ± 4.0 years. The Pras, Mor, and ISSF scores were inconsistent with each other, and there was poor fit between them (generalised Kappa: 0.140 ± 0.029; P < .001). In the receiver operating characteristic (ROC) analysis performed by accepting the clinician's opinion as the gold standard, the ISSF was found to be more sensitive and specific than the other two systems. Evaluation of disease severity according to the ISSF in paediatric patients is more sensitive and specific than the Pras and Mor scoring systems.
Substances chimiques
Colchicine
SML2Y3J35T
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e14678Informations de copyright
© 2021 John Wiley & Sons Ltd.
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