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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...
Autores principales: | , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2021
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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. |
format | Online Article Text |
id | pubmed-7811395 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
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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