Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients

Abstract Background There have been numerous classification systems to diagnose corresponding myositis subtypes and select appropriate therapeutic measures. However, the lack of a broad consensus on diagnostic criteria has led to clinical uncertainties. The objective of this study was to compare two...

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Autores principales: Jan Zoske, Udo Schneider, Elise Siegert, Felix Kleefeld, Corinna Preuße, Werner Stenzel, Katrin Hahn
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Publicado: BMC 2021
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spelling oai:doaj.org-article:c39b7f74cf4344b4baca0950adb8d1d12021-11-21T12:04:48ZPerformance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients10.1186/s42466-021-00159-42524-3489https://doaj.org/article/c39b7f74cf4344b4baca0950adb8d1d12021-11-01T00:00:00Zhttps://doi.org/10.1186/s42466-021-00159-4https://doaj.org/toc/2524-3489Abstract Background There have been numerous classification systems to diagnose corresponding myositis subtypes and select appropriate therapeutic measures. However, the lack of a broad consensus on diagnostic criteria has led to clinical uncertainties. The objective of this study was to compare two commonly used dermatomyositis-classification systems regarding their clinical practicability and to point out their specific advantages and disadvantages. Methods This study included 30 patients diagnosed with dermatomyositis at the Charité university hospital, Berlin, Germany from 2010 to 2017. Patient files with complete data and defined historical classifications were enrolled and ENMC (2003) and EULAR/ACR (2017) criteria retrospectively applied. Results According to the ENMC approach, 14 patients were classified as "definite" and 12 as "probable" dermatomyositis. One patient exhibited an "amyopathic dermatomyositis" and three a "DM without dermatitis". Regarding the criteria probability of the EULAR/ACR set, 16 patients had a "high", 13 a "medium" and one a "low probability". There was a significant difference (p = 0.004) between the subclasses of the ENMC in relation to the EULAR/ACR score. The agreement between the classification probabilities of "definite/high" (κ = 0.400) and "possible/medium" (κ = 0.324) was fair. Conclusions It is important to find a consensus among the medical disciplines involved and to establish a structured procedure. Future studies with newer approaches are warranted to conclusively decide which system to use for the physician.Jan ZoskeUdo SchneiderElise SiegertFelix KleefeldCorinna PreußeWerner StenzelKatrin HahnBMCarticleDermatomyositisENMCEULAR/ACRIdiopathic inflammatory myositisMuscle biopsyNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571Neurology. Diseases of the nervous systemRC346-429ENNeurological Research and Practice, Vol 3, Iss 1, Pp 1-9 (2021)
institution DOAJ
collection DOAJ
language EN
topic Dermatomyositis
ENMC
EULAR/ACR
Idiopathic inflammatory myositis
Muscle biopsy
Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
Neurology. Diseases of the nervous system
RC346-429
spellingShingle Dermatomyositis
ENMC
EULAR/ACR
Idiopathic inflammatory myositis
Muscle biopsy
Neurosciences. Biological psychiatry. Neuropsychiatry
RC321-571
Neurology. Diseases of the nervous system
RC346-429
Jan Zoske
Udo Schneider
Elise Siegert
Felix Kleefeld
Corinna Preuße
Werner Stenzel
Katrin Hahn
Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients
description Abstract Background There have been numerous classification systems to diagnose corresponding myositis subtypes and select appropriate therapeutic measures. However, the lack of a broad consensus on diagnostic criteria has led to clinical uncertainties. The objective of this study was to compare two commonly used dermatomyositis-classification systems regarding their clinical practicability and to point out their specific advantages and disadvantages. Methods This study included 30 patients diagnosed with dermatomyositis at the Charité university hospital, Berlin, Germany from 2010 to 2017. Patient files with complete data and defined historical classifications were enrolled and ENMC (2003) and EULAR/ACR (2017) criteria retrospectively applied. Results According to the ENMC approach, 14 patients were classified as "definite" and 12 as "probable" dermatomyositis. One patient exhibited an "amyopathic dermatomyositis" and three a "DM without dermatitis". Regarding the criteria probability of the EULAR/ACR set, 16 patients had a "high", 13 a "medium" and one a "low probability". There was a significant difference (p = 0.004) between the subclasses of the ENMC in relation to the EULAR/ACR score. The agreement between the classification probabilities of "definite/high" (κ = 0.400) and "possible/medium" (κ = 0.324) was fair. Conclusions It is important to find a consensus among the medical disciplines involved and to establish a structured procedure. Future studies with newer approaches are warranted to conclusively decide which system to use for the physician.
format article
author Jan Zoske
Udo Schneider
Elise Siegert
Felix Kleefeld
Corinna Preuße
Werner Stenzel
Katrin Hahn
author_facet Jan Zoske
Udo Schneider
Elise Siegert
Felix Kleefeld
Corinna Preuße
Werner Stenzel
Katrin Hahn
author_sort Jan Zoske
title Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients
title_short Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients
title_full Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients
title_fullStr Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients
title_full_unstemmed Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patients
title_sort performance of enmc and eular/acr classification systems applied to a single tertiary center cohort of dermatomyositis patients
publisher BMC
publishDate 2021
url https://doaj.org/article/c39b7f74cf4344b4baca0950adb8d1d1
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