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...
Main Authors: | , , , , , , |
---|---|
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 |
_version_ | 1819199024207495168 |
---|---|
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. |
first_indexed | 2024-12-23T03:09:45Z |
format | Article |
id | doaj.art-c39b7f74cf4344b4baca0950adb8d1d1 |
institution | Directory Open Access Journal |
issn | 2524-3489 |
language | English |
last_indexed | 2024-12-23T03:09:45Z |
publishDate | 2021-11-01 |
publisher | BMC |
record_format | Article |
series | Neurological Research and Practice |
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 |
work_keys_str_mv | AT janzoske performanceofenmcandeularacrclassificationsystemsappliedtoasingletertiarycentercohortofdermatomyositispatients AT udoschneider performanceofenmcandeularacrclassificationsystemsappliedtoasingletertiarycentercohortofdermatomyositispatients AT elisesiegert performanceofenmcandeularacrclassificationsystemsappliedtoasingletertiarycentercohortofdermatomyositispatients AT felixkleefeld performanceofenmcandeularacrclassificationsystemsappliedtoasingletertiarycentercohortofdermatomyositispatients AT corinnapreuße performanceofenmcandeularacrclassificationsystemsappliedtoasingletertiarycentercohortofdermatomyositispatients AT wernerstenzel performanceofenmcandeularacrclassificationsystemsappliedtoasingletertiarycentercohortofdermatomyositispatients AT katrinhahn performanceofenmcandeularacrclassificationsystemsappliedtoasingletertiarycentercohortofdermatomyositispatients |