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

Demirkaya, Erkan; Açıkel, Cengizhan; Hashkes, Philip J.; Gattorno, Marco; Gül, Ahmet; Özdoğan, Huri; Türker, Türker; Karadağ, Ömer; Livneh, Avi; Ben-Chétrit, Eldad; Özen, Seza; Vasculitis, Fmf Arthritis

doi:10.1136/annrheumdis-2015-208671

Cited by 88 publications

(59 citation statements)

References 27 publications

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“…Disease severity was grouped as severe (≥6 points), intermediate (3-5 points), or mild (≤2 points). 12 Growth parameters including weight and height were investigated; thereafter, standard deviation (SD) scores were calculated by anthropometric references in Turkish children. 13 The study protocol was approved by the Çukurova University Faculty of Medicine Ethics Committee (number: 78/68; date: 01 June 2018).…”

Section: Methodsmentioning

confidence: 99%

Disease Severity and Genotype Affect Physical Growth in Children With Familial Mediterranean Fever

et al. 2019

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Objectives: This study aims to analyze the growth parameters in children with familial Mediterranean fever (FMF) according to disease characteristics including genotype and disease severity by a recently validated tool in relatively more patients. Patients and methods: This retrospective study included 126 patients with FMF (70 males, 56 females; mean age 7.3±3.6 years; range, 4.1 to 18 years). MEditerranean FeVer (MEFV) gene analysis was performed with a molecular diagnostics tool by using a next-generation sequencing platform. Disease severity was determined for the first visit by the validated tool in children, international severity scoring system for FMF. Growth parameters including weight and height were investigated after standard deviation (SD) scores were calculated by anthropometric references in Turkish children. Results: Median follow-up duration was 74.7 months (range, 7.5 to 169 months). Ninety-three patients (73.8%) had at least one M694V mutation in MEFV gene. Six patients (4.8%) had severe disease, 58 (46%) had intermediate severity, and 62 (49.2%) had mild disease. Mean height SD score was significantly lower at last visit than before colchicine treatment. Initial and last height and weight SD scores were lower in patients with at least one M694V mutation than those without. However, the difference was statistically significant for only initial height SD score. We also found statistically significant lower initial height, final height, and weight SD scores in patients with intermediate severity-severe disease activity than mild disease. Conclusion: We advise physicians to score disease severity prospectively and pay attention to patients with intermediate severity-severe disease to avoid growth disturbances.

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Section: Methodsmentioning

confidence: 99%

Disease Severity and Genotype Affect Physical Growth in Children With Familial Mediterranean Fever

et al. 2019

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“…The severity of disease was evaluated following the instructions of the FMF Arthritis Vasculitis and Orphan disease Research in paediatric rheumatology (FAVOR) by a recently validated questionnaire: international severity scoring system for FMF (ISSF) [9]. abdominal pain, chest pain), skin manifestations, quality of life (QoL).…”

Section: Clinical Featuresmentioning

confidence: 99%

“…abdominal pain, chest pain), skin manifestations, quality of life (QoL). The severity of disease was evaluated following the instructions of the FMF Arthritis Vasculitis and Orphan disease Research in paediatric rheumatology (FAVOR) by a recently validated questionnaire: international severity scoring system for FMF (ISSF) [9]. Symptomatic patients were subsequently treated with colchicine (Colchicina Lirca â , Acarpia Lda, Portugal) to prevent the attacks and complications of FMF.…”

Section: Clinical Featuresmentioning

confidence: 99%

A novel cluster of patients with Familial Mediterranean Fever (FMF) in southern Italy

Bonfrate

Scaccianoce²,

Palasciano

et al. 2017

Eur J Clin Investigation

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“…These authors have also collaborated with colleagues from Turkey and Italy [6] in defining a severity score for FMF which will serve as a useful tool in assessing the utility of therapeutic interventions in clinical trials in both children and adults.…”

Section: Research In the Middle Eastmentioning

confidence: 99%

Rheumatology in the Middle East in 2017: clinical challenges and research

et al. 2017

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The World Health Organization (WHO) has ranked musculoskeletal diseases among the top ten leading causes of disability in the Middle East. The situation in the Middle East is unique as, although seventeen countries are geographical connected, there is considerable variability in the standard of rheumatology care and research between the countries. There are several factors contributing to this variability: allocated government resources to healthcare services, country demographics, the implemented healthcare system, the number of qualified rheumatologist, political stability, and the immigration to and from these countries.

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

Abstract: The 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.

Cited by 88 publications

References 27 publications

Disease Severity and Genotype Affect Physical Growth in Children With Familial Mediterranean Fever

Disease Severity and Genotype Affect Physical Growth in Children With Familial Mediterranean Fever

A novel cluster of patients with Familial Mediterranean Fever (FMF) in southern Italy

Rheumatology in the Middle East in 2017: clinical challenges and research

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