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Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey

BACKGROUND: Although not validated fully, recommendations are present for diagnosis, screening and treatment modalities of patients with familial Mediterranean fever (FMF). OBJECTIVE: To review the current practices of clinicians regarding FMF and reveal their adherence to consensus guidelines. METH...

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Autores principales: Kavrul Kayaalp, Gülsah, Sozeri, Betül, Sönmez, Hafize Emine, Demir, Ferhat, Cakan, Mustafa, Oztürk, Kübra, Karadag, Serife Gül, Otar Yener, Gülcin, Ozdel, Semanur, Baglan, Esra, Celikel, Elif, Sahin, Nihal, Gezgin Yildirim, Deniz, Eker Omeroglu, Rukiye, Aktay Ayaz, Nuray
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7811395/
https://www.ncbi.nlm.nih.gov/pubmed/33454820
http://dx.doi.org/10.1007/s00296-020-04776-1
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author Kavrul Kayaalp, Gülsah
Sozeri, Betül
Sönmez, Hafize Emine
Demir, Ferhat
Cakan, Mustafa
Oztürk, Kübra
Karadag, Serife Gül
Otar Yener, Gülcin
Ozdel, Semanur
Baglan, Esra
Celikel, Elif
Sahin, Nihal
Gezgin Yildirim, Deniz
Eker Omeroglu, Rukiye
Aktay Ayaz, Nuray
author_facet Kavrul Kayaalp, Gülsah
Sozeri, Betül
Sönmez, Hafize Emine
Demir, Ferhat
Cakan, Mustafa
Oztürk, Kübra
Karadag, Serife Gül
Otar Yener, Gülcin
Ozdel, Semanur
Baglan, Esra
Celikel, Elif
Sahin, Nihal
Gezgin Yildirim, Deniz
Eker Omeroglu, Rukiye
Aktay Ayaz, Nuray
author_sort Kavrul Kayaalp, Gülsah
collection PubMed
description BACKGROUND: Although not validated fully, recommendations are present for diagnosis, screening and treatment modalities of patients with familial Mediterranean fever (FMF). OBJECTIVE: To review the current practices of clinicians regarding FMF and reveal their adherence to consensus guidelines. METHODS: Fifteen key points selected regarding the diagnosis and management of FMF were assessed by 14 paediatric rheumatologists with a three-round modified Delphi panel. RESULTS: Consensus was reached on the following aspects: genetic analysis should be ordered to all patients when clinical findings support FMF, but its result is not decisive alone. In the absence of clinical features, colchicine should be commenced when two pathogenic alleles and family history of amyloidosis are present. Serum amyloid A testing at each visit is recommended in patients resistant to colchicine, with subclinical inflammation and family history of amyloidosis. Consensus was reached on both the definition of colchicine resistance and starting biologic in resistant cases. Cost, efficiency, ease of use, treatment adherence, accessibility and emergence of adverse events are the factors affecting the choice of biologic agents. In patients without any attack and evidence of subclinical inflammation within the last 6 months following initiation of biologics, treatment dose intervals can be prolonged. CONCLUSION: A consensus was achieved regarding the routine diagnosis and screening and treatment of FMF patients. The definition of colchicine resistance was made and a protocol was created for prolongation of treatment intervals of biologic agents. We anticipate that the results of the study reveal real-life data on the approach to patients in clinical practice. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00296-020-04776-1.
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spelling pubmed-78113952021-01-18 Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey Kavrul Kayaalp, Gülsah Sozeri, Betül Sönmez, Hafize Emine Demir, Ferhat Cakan, Mustafa Oztürk, Kübra Karadag, Serife Gül Otar Yener, Gülcin Ozdel, Semanur Baglan, Esra Celikel, Elif Sahin, Nihal Gezgin Yildirim, Deniz Eker Omeroglu, Rukiye Aktay Ayaz, Nuray Rheumatol Int Observational Research BACKGROUND: Although not validated fully, recommendations are present for diagnosis, screening and treatment modalities of patients with familial Mediterranean fever (FMF). OBJECTIVE: To review the current practices of clinicians regarding FMF and reveal their adherence to consensus guidelines. METHODS: Fifteen key points selected regarding the diagnosis and management of FMF were assessed by 14 paediatric rheumatologists with a three-round modified Delphi panel. RESULTS: Consensus was reached on the following aspects: genetic analysis should be ordered to all patients when clinical findings support FMF, but its result is not decisive alone. In the absence of clinical features, colchicine should be commenced when two pathogenic alleles and family history of amyloidosis are present. Serum amyloid A testing at each visit is recommended in patients resistant to colchicine, with subclinical inflammation and family history of amyloidosis. Consensus was reached on both the definition of colchicine resistance and starting biologic in resistant cases. Cost, efficiency, ease of use, treatment adherence, accessibility and emergence of adverse events are the factors affecting the choice of biologic agents. In patients without any attack and evidence of subclinical inflammation within the last 6 months following initiation of biologics, treatment dose intervals can be prolonged. CONCLUSION: A consensus was achieved regarding the routine diagnosis and screening and treatment of FMF patients. The definition of colchicine resistance was made and a protocol was created for prolongation of treatment intervals of biologic agents. We anticipate that the results of the study reveal real-life data on the approach to patients in clinical practice. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s00296-020-04776-1. Springer Berlin Heidelberg 2021-01-16 2022 /pmc/articles/PMC7811395/ /pubmed/33454820 http://dx.doi.org/10.1007/s00296-020-04776-1 Text en © The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature 2021 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.
spellingShingle Observational Research
Kavrul Kayaalp, Gülsah
Sozeri, Betül
Sönmez, Hafize Emine
Demir, Ferhat
Cakan, Mustafa
Oztürk, Kübra
Karadag, Serife Gül
Otar Yener, Gülcin
Ozdel, Semanur
Baglan, Esra
Celikel, Elif
Sahin, Nihal
Gezgin Yildirim, Deniz
Eker Omeroglu, Rukiye
Aktay Ayaz, Nuray
Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey
title Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey
title_full Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey
title_fullStr Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey
title_full_unstemmed Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey
title_short Adherence to best practice consensus guidelines for familial Mediterranean fever: a modified Delphi study among paediatric rheumatologists in Turkey
title_sort adherence to best practice consensus guidelines for familial mediterranean fever: a modified delphi study among paediatric rheumatologists in turkey
topic Observational Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7811395/
https://www.ncbi.nlm.nih.gov/pubmed/33454820
http://dx.doi.org/10.1007/s00296-020-04776-1
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