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Performance of the new 'Eurofever/PRINTO classification criteria' in FMF patients
Erdal Sag1, Dilara Demirel2, Selcan Demir1
1Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University, Ankara, Turkey.
Objective:
Recently a new set of criteria proposed for the classification of auto inflammatory recurrent fevers including familial Mediterranean Fever (FMF). We aimed to compare the sensitivity and specificity of the new Eurofever/PRINTO classification criteria with those of the Tel Hashomer and Yalcinkaya-Ozen criteria.
Methods:
151 consecutive FMF patients between February and May 2019 who were followed at Hacettepe University Department of Pediatric Rheumatology were included in this study. A group of 82 patients with periodic fever 66 periodic fever, aphthosis, pharyngitis and adenitis syndrome (PFAPA), nine cryopyrin-associated periodic syndrome (CAPS) and seven mevalonate kinase deficiency/hyperimmunoglobulin D syndrome (MKD/HIDS) patients) served as controls. GraphPad 6.0 was used for statistical analysis.
Results:
Three different classification criteria were analyzed in 151 FMF patients with a median age at diagnosis of 5 years and in 82 controls with a median age at diagnosis of 3 years. The sensitivity of the new Eurofever/PRINTO criteria (96%) was highest (Tel Hashomer criteria-88.4% and Yalcinkaya-Ozen criteria-93.4%). However, the specificity of these criteria (73.1%) was lowest (Tel Hashomer criteria-92.6% and Yalcinkaya-Ozen criteria-84.1%). The new Eurofever/PRINTO criteria achieved the highest sensitivity (100%) in biallelic exon 10 mutation patients (Tel Hashomer criteria-87.4% and Yalcinkaya-Ozen criteria-94.2%). However, the new set had the lowest sensitivity (88.2%) in heterozygote exon 10 mutation patients (Tel Hashomer criteria 94.1% and Yalcinkaya-Ozen criteria 94.1%).
Conclusion:
In this Turkish cohort, the new Eurofever/PRINTO criteria have a better sensitivity but lower specificity with higher misclassifications than other two well-known criteria. The combination of clinical manifestations with genotype increased the sensitivity. The lower specificity may be due to the high carrier rate in our population. Although the ethnicity information lowers the specificity, 'clinical-only' criteria set may still guide the clinician to perform appropriate genetic testing in patients with recurrent fever.
Insights
The new Eurofever/PRINTO criteria for familial Mediterranean Fever (FMF) show higher sensitivity but lower specificity compared to older methods in a Turkish cohort. Combining clinical data with genetic testing improves accuracy for diagnosing recurrent fevers.
Area of Science:
- Rheumatology
- Genetics
- Pediatrics
Background:
- Auto-inflammatory recurrent fevers, including familial Mediterranean Fever (FMF), require accurate classification for effective management.
- New diagnostic criteria, such as the Eurofever/PRINTO classification, have been proposed to improve FMF diagnosis.
- Comparing these new criteria with established ones like Tel Hashomer and Yalcinkaya-Ozen is crucial for clinical application.
Purpose of the Study:
- To compare the sensitivity and specificity of the new Eurofever/PRINTO classification criteria against the Tel Hashomer and Yalcinkaya-Ozen criteria in a cohort of FMF patients.
- To evaluate the diagnostic performance of these criteria in a Turkish population.
Main Methods:
- A study was conducted on 151 consecutive FMF patients and 82 controls with other periodic fever syndromes.
- The Tel Hashomer, Yalcinkaya-Ozen, and Eurofever/PRINTO classification criteria were applied to all patients.
- Statistical analysis was performed using GraphPad 6.0.
Main Results:
- The Eurofever/PRINTO criteria demonstrated the highest sensitivity (96%) but the lowest specificity (73.1%) among the evaluated criteria.
- Sensitivity was highest (100%) in patients with biallelic exon 10 mutations using Eurofever/PRINTO criteria.
- Specificity was notably lower in the Turkish cohort, potentially due to a high carrier rate.
Conclusions:
- In the studied Turkish cohort, the Eurofever/PRINTO criteria offer improved sensitivity but reduced specificity for diagnosing FMF compared to Tel Hashomer and Yalcinkaya-Ozen criteria.
- Integrating clinical manifestations with genotypic information enhances diagnostic sensitivity.
- Despite lower specificity, the 'clinical-only' aspect of the Eurofever/PRINTO criteria can guide clinicians toward appropriate genetic testing for recurrent fever syndromes.
