Development and initial validation of international severity scoring system for familial Mediterranean fever (ISSF)

@article{Demirkaya2016DevelopmentAI,
  title={Development and initial validation of international severity scoring system for familial Mediterranean fever (ISSF)},
  author={Erkan Demirkaya and Cengizhan Açikel and Philip J. Hashkes and Marco Gattorno and Ahmet Gul and Huri Ozdogan and Turker Turker and Omer Karadag and Avi Livneh and Eldad Ben-Chetrit and Seza Ozen},
  journal={Annals of the Rheumatic Diseases},
  year={2016},
  volume={75},
  pages={1051 - 1056}
}
Objective To 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. Methods Candidate 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… 
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58 Citations
Highly Influential Citations
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Background Citations
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Methods Citations
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58 Citations

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Citation Type

How to define disease severity accurately in patients with familial Mediterranean fever
TLDR
It is suggested that it is important to use validated outcome measures in any type of the study to achieve standardization and quantitative definition of patient’s well-being in the era of patient care as well as clinical research.
Disease Severity and Genotype Affect Physical Growth in Children With Familial Mediterranean Fever.
TLDR
The growth parameters in children with familial Mediterranean fever according to disease characteristics including genotype and disease severity by a recently validated tool by using a next-generation sequencing platform are analyzed to avoid growth disturbances.
Is the performance of the international severity scoring system for familial mediterranean fever in children better than other scoring systems?
TLDR
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.
Familial Mediterranean Fever (FMF): a single centre retrospective study in Amsterdam.
TLDR
Familial Mediterranean Fever in Amsterdam is diagnosed in relatively young patients and the delay to diagnosis is 8.2 years, suggesting that ancestry is more important than environment.
Evaluation of the international severity score for FMF (ISSF) scores in Turkish children diagnosed with FMF: a single-center experience
TLDR
The newly developed international severity score for FMF (ISSF) appears as a suitable and effective tool for the physicians in the follow-up of the disease severity in pediatric FMF patients and the addition of pathogenic mutations and inheritance model to the ISSF scoring system as important determinants of disease severity can be evaluated in future studies.
Factors associated with damage in patients with familial Mediterranean fever.
TLDR
This study is the first to apply comprehensive damage indexes to FMF patients and identified predictors of damage, including factors linked to a severe FMF phenotype, which justify the concerns about musculoskeletal pain and might point to the need for re-evaluation of ADDI.
The Influence of Concomitant Disorders on Disease Severity of Familial Mediterranean Fever in Children.
TLDR
It is indicated that concomitant diseases, particularly JIA, influence FMF severity and it may be beneficial to focus on diagnosis and treatment of comorbid inflammatory diseases, which may worsen the course of FMF.
Compliance of Familial Mediterranean Fever Patients With Regular Follow-up Visits and Associated Factors.
TLDR
A family history of FMF in parents, the absence of a family history in siblings, treatment with biologic agents, concomitant medication use, multisite involvement during FMF attacks, and treatment satisfaction were independent predictors of visit compliance.
Is age associated with disease severity and compliance to treatment in children with familial Mediterranean fever?
TLDR
The disease characteristics of FMF may differ between patients with different age at symptom onset, and treatment compliance which is critical for prevention of amyloidosis in FMF should be questioned especially in adolescent patients.
Familial Mediterranean fever: Health-related quality of life and associated variables in a national cohort
TLDR
Female sex, smoking, lower educational status, more severe disease, fatigue, and functional impairment were associated with poor QoL in patients with Familial Mediterranean Fever.
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