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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Main Authors: Jan Zoske, Udo Schneider, Elise Siegert, Felix Kleefeld, Corinna Preuße, Werner Stenzel, Katrin Hahn
Format: Article
Language:English
Published: BMC 2021-11-01
Series:Neurological Research and Practice
Subjects:
Online Access:https://doi.org/10.1186/s42466-021-00159-4
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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
collection DOAJ
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.
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spelling doaj.art-c39b7f74cf4344b4baca0950adb8d1d12022-12-21T18:02:16ZengBMCNeurological Research and Practice2524-34892021-11-01311910.1186/s42466-021-00159-4Performance of ENMC and EULAR/ACR classification systems applied to a single tertiary center cohort of dermatomyositis patientsJan Zoske0Udo Schneider1Elise Siegert2Felix Kleefeld3Corinna Preuße4Werner Stenzel5Katrin Hahn6Department of Periodontology, Oral Medicine and Oral Surgery, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, and Berlin Institute of HealthDepartment of Rheumatology and Clinical Immunology, Charité – Universitätsmedizin BerlinDepartment of Rheumatology and Clinical Immunology, Charité – Universitätsmedizin BerlinDepartment of Neurology, Charité - Universitätsmedizin BerlinDepartment of Neuropathology, Charité - Universitätsmedizin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, and Berlin Institute of Health (BIH)Department of Neuropathology, Charité - Universitätsmedizin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, and Berlin Institute of Health (BIH)Department of Neurology, Charité - Universitätsmedizin BerlinAbstract 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.https://doi.org/10.1186/s42466-021-00159-4DermatomyositisENMCEULAR/ACRIdiopathic inflammatory myositisMuscle biopsy
spellingShingle 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
Neurological Research and Practice
Dermatomyositis
ENMC
EULAR/ACR
Idiopathic inflammatory myositis
Muscle biopsy
title 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_short 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
topic Dermatomyositis
ENMC
EULAR/ACR
Idiopathic inflammatory myositis
Muscle biopsy
url https://doi.org/10.1186/s42466-021-00159-4
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