Clinicopathological Classification of Dermatomyositis
- Version 239
- Download 14
- File Size 4.00 KB
- File Count 1
- Create Date 14 December 2018
- Last Updated 27 May 2020
Location: Hoofddorp, The Netherlands
Title: The 239th ENMC workshop on "The Clinicopathological Classification of Dermatomyositis”
Date: 14-16 December 2018
Organisers: Dr Y. Allenbach (France), Prof. O. Benveniste (France), Dr A. Mammen (U.S.A), Prof. W. Stenzel (Germany)
Translations of this report:
Spanish by Dr A. Selva O Callaghan
Dutch by Ms. I. de Groot and Prof. M. de Visser
Czech by Prof. J. Vencovský
Deutsch by S. Schlüter und Dr. A. v. Moers
Participants: Dr Y. Allenbach (France), Prof. O. Benveniste (France), Prof. J. de Bleecker (Belgium), Dr O. Boyer (France), Prof. L. Casciola-Rosen (U.S.A.), Dr L. Christopher-Stine (U.S.A), Dr J. Damoiseaux (The Netherlands), Prof. M. Fujimoto (Japan), Dr C. Gitiaux (France), Ms. I. de Groot (The Netherlands), Dr J. Lamb (United Kingdom), Dr O. Landon-Cardinal (Canada), Prof. I. Lundberg (Sweden), Dr A. Mammen (U.S.A.), Dr I. Nishino (Japan), Dr J. Radke (Germany), Dr A. Selva-O´Callaghan (Spain), Prof. W. Stenzel (Germany), Prof. J. Vencovksi (Czech Republic), Prof. M. de Visser (The Netherlands), Dr G. Wang (China), Prof. L. Wedderburn (United Kingdom) and Dr V. Werth (U.S.A.)
The 239th ENMC workshop on the “Clinicopathological Classification of Dermatomyositis” brought together experts in the field of myositis from Europe, Japan, China, Canada and the USA. Neurologists, Rheumatologists, Dermatologists, Neuropathologists and Myopathologists discussed and renewed the diagnostic criteria of dermatomyositis (DM) based on recent advances in the field. All experts agreed on the importance of mandatory testing of myositis specific autoantibodies (MSA, e.g. TIF-1γ, Mi-2, NXP2, MDA5 and SAE) in the diagnostic workup of DM patients. Furthermore, they emphasized the importance of autoantibodies to define certain subtypes of DM, which may have a characteristic clinical, pathological and even histological appearance. If the clinical presentation of a patient is classical, consisting of characteristic, DM-associated skin changes (e.g. DM-like rash), muscle weakness and detection of any MSA, the diagnosis of DM may be made, even without a muscle biopsy. Moreover, the finding of characteristic skin changes, clinically and in a skin biopsy, in combination with MSA but without muscle affection is also compatible with the diagnosis of DM. A more detailed definition of DM-like skin changes and histological criteria seen in the skin biopsy of an affected skin area in a DM patient will soon be published by an international expert team of dermatologists. It was clear to all experts that the muscle biopsy was and still is a very valuable tool and the gold standard in the diagnostic routine. Novel, specialized immunohistochemical stains further increase the certainty of a correct histological diagnosis. Especially in TIF-1γ positive DM patients, the muscle biopsy combined with the patients´ age may help to stratify patients concerning their risk of already having or developing a malignancy. It became clear, that TIF-1γ should not be misinterpreted as a bona fide tumor marker. However older patients with TIF-1γ positive DM definitely need an oncological screening in routine care since cancer risk is prominently elevated.
Additionally, the classification of the Anti-synthetase syndrome (ASS) was a matter of debate. Recent scientific data presented in the ENMC workshop clearly highlighted the differences of DM and ASS. All experts agreed on ASS being a distinct subtype, pathophysiologically clearly different from DM. Nevertheless, ASS can in very rare cases be considered as DM-related.
A full report is published in Neuromuscular Disorders (pdf).
ENMC
Lt. generaal van Heutszlaan 6
3743 JN BAARN
The Netherlands
+ 31- 35-5480481
enmc@enmc.org
We may request cookies to be set on your device. We use cookies to let us know when you visit our websites, how you interact with us, to enrich your user experience, and to customize your relationship with our website.
Click on the different category headings to find out more. You can also change some of your preferences. Note that blocking some types of cookies may impact your experience on our websites and the services we are able to offer.
These cookies are strictly necessary to provide you with services available through our website and to use some of its features.
Because these cookies are strictly necessary to deliver the website, refusing them will have impact how our site functions. You always can block or delete cookies by changing your browser settings and force blocking all cookies on this website. But this will always prompt you to accept/refuse cookies when revisiting our site.
We fully respect if you want to refuse cookies but to avoid asking you again and again kindly allow us to store a cookie for that. You are free to opt out any time or opt in for other cookies to get a better experience. If you refuse cookies we will remove all set cookies in our domain.
We provide you with a list of stored cookies on your computer in our domain so you can check what we stored. Due to security reasons we are not able to show or modify cookies from other domains. You can check these in your browser security settings.
These cookies collect information that is used either in aggregate form to help us understand how our website is being used or how effective our marketing campaigns are, or to help us customize our website and application for you in order to enhance your experience.
If you do not want that we track your visit to our site you can disable tracking in your browser here:
We also use different external services like Google Webfonts, Google Maps, and external Video providers. Since these providers may collect personal data like your IP address we allow you to block them here. Please be aware that this might heavily reduce the functionality and appearance of our site. Changes will take effect once you reload the page.
Google Webfont Settings:
Google Map Settings:
Google reCaptcha Settings:
Vimeo and Youtube video embeds:
The following cookies are also needed - You can choose if you want to allow them:
You can read about our cookies and privacy settings in detail on our Privacy Policy Page.
Privacy Policy