ArticleAnnals of the rheumatic diseases2016
Development and initial validation of international severity scoring system for familial Mediterranean fever (ISSF).
Article in Annals of the rheumatic diseases, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03563300 (Gluten-related Disorders in Patients Affected With Familial Mediterranean Fever), which is not on this map. Cited by 70 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Gluten-related Disorders in Patients Affected With Familial Mediterranean Fever
Who cites it
70 citing papers in PubMed, 1 synthesis or guideline pooled it, 129 citations in OpenAlex.
- [Results of the systematic literature search as basis for the "Evidence-based treatment recommendations for familial Mediterranean fever patients with insufficient response or intolerability to colchicine" of the Society for Pediatric and Adolescent Rheumatology and the German Society for Rheumatology].Zeitschrift fur Rheumatologie · 2020Pooled it
- Musculoskeletal manifestations occur predominantly in patients with later-onset familial Mediterranean fever: Data from a multicenter, prospective national cohort study in Japan.Arthritis research & therapy · 2018Trial
- Evaluation of subclinical organ involvement with ultrasound elastography in childhood familial Mediterranean fever: relationships with disease activity, inflammatory markers, colchicine response, and genotype.European journal of pediatrics · 2026Article
- Long-term clinical course of pediatric familial Mediterranean fever with heterozygous pathogenic MEFV exon 10 variants.European journal of pediatrics · 2026Article
- Association of Hemoglobin Level and Diagnostic Delay with Left Atrial Volume Index in Patients with Familial Mediterranean Fever: A Cross-Sectional Observational Study.Journal of clinical medicine · 2026Article
- Effectiveness of colchicine preparation switching in pediatric colchicine-resistant familial Mediterranean fever: a real-world study.European journal of pediatrics · 2026Article
- Exertional leg pain is associated with ıncreased disease activity and musculoskeletal ınvolvement, but not MEFV genotype, in familial Mediterranean fever.Irish journal of medical science · 2026Article
- Sleep Quality and Depressive Symptoms in Clinically Stable Pediatric Familial Mediterranean Fever: Associations with Disease Severity and Developmental Factors.Children (Basel, Switzerland) · 2026Article
- Discontinuing Colchicine in Pediatric Familial Mediterranean Fever: Real-Life Experience from a Long-Term Follow-Up Cohort.Paediatric drugs · 2026Article
- From diagnosis to treatment response: predicting FMF50 response using PREDICT-crFMF and TURPAID scores in children with Familial Mediterranean Fever: a retrospective cohort study.Clinical rheumatology · 2026Article
- Clinical Association of Pan-Immune-Inflammation Value with MEFV Mutation Burden and Amyloidosis in Adults with Familial Mediterranean Fever: A Retrospective Cohort Study.Journal of clinical medicine · 2026Article
- Subclinical inflammation in pediatric familial Mediterranean fever: predictors and follow-up results of a retrospective single-center cohort study.Clinical rheumatology · 2026Article
- Growth differentiation factor-15 as a potential biomarker in subclinical inflammation in familial mediterranean fever.Clinical rheumatology · 2026Article
- Evidence of the role of Midkine and Pleiotrophin in the pathogenesis of Familial Mediterranean Fever.European journal of pediatrics · 2026Article
- Ultrasonographic evaluation of FMF patients with exertional leg pain: an overlooked component of the disease.Rheumatology (Oxford, England) · 2026Article
- Exertional leg pain in pediatric familial mediterranean fever: clinical associations and implications for treatment.European journal of pediatrics · 2026Article
- Comparative analysis of exon 10 and non-exon 10 variants in children with familial mediterranean fever: a retrospective cohort study.European journal of pediatrics · 2026Article
- Retinal changes in children with Familial Mediterranean Fever: the effect of chronic subclinical inflammation.Pediatric research · 2026Article
- Practical considerations for monitoring patients with familial Mediterranean fever: review of evidence and expert opinion.Frontiers in immunology · 2026Review
- Growth and pubertal development in children with familial Mediterranean fever under colchicine therapy.European journal of pediatrics · 2025Article
10 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
12 authors at 7 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo develop widely accepted international severity score for children and adult patients with familial Mediterranean fever (FMF) that can be easily applied, in research and clinical practice.
methodsCandidate severity criteria were suggested by several FMF expert physicians. After three rounds of Delphi survey, the candidate criteria, defined by the survey, were discussed by experts in a consensus meeting. Each expert brought data of clinical manifestations, laboratory findings and physician's global assessments (PGAs) of minimum 20 patients from their centres. We used the PGAs for disease severity as a gold standard. Logistic regression analysis was used to evaluate the predicting value of each item, and receiver operating characteristic curve analysis was performed to demonstrate the success of the criteria set.
resultsA total of 281 patients consist of 162 children and 119 adults with FMF were enrolled and available for validity analysis: Nine domains were included in the final core set of variables for the evaluation of disease severity in FMF. The International Severity Score for FMF (ISSF) may reach a maximum of 10 if all items are maximally scored. The threshold values to determine: severe disease ≥6, intermediate disease 3-5, mild disease ≤2. Area under the curve was calculated as 0.825 for this set in the whole group.
conclusionsThe initial validity of ISSF both in children and adult with FMF was demonstrated. We anticipate that it will provide a robust tool to objectively define disease severity for clinical trials, future research as well as for therapeutic decisions in managing patients with FMF.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.