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Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry
RM. Hum, JB. Lilleker, JA. Lamb, AGS. Oldroyd, G. Wang, LR. Wedderburn, LP. Diederichsen, J. Schmidt, MG. Danieli, P. Oakley, Z. Griger, T. Nguyen Thi Phuong, C. Kodishala, M. Vazquez-Del Mercado, H. Andersson, B. De Paepe, JL. De Bleecker, B....
Language English Country England, Great Britain
Document type Journal Article, Comparative Study
Grant support
Association Francaise Contre Les Myopathies
LSH-018661
he European Union Sixth Framework Programme
European Science Foundation
Research Networking Programme European Myositis Network
K2014-52X-14045-14-3
Swedish Research Council
Stockholm County Council and Karolinska Institutet
MR/N003322/1
Medical Research Council - United Kingdom
18474
Arthritis Research UK - United Kingdom
00023728
Project for Conceptual Development of Research Organization
Ministry of Health in the Czech Republic
National Institute for Health Research
NIHR203308
Manchester Biomedical Research Centre
National Institute for Health Research
GOSH Biomedical Research Centre
Department of Health and Social Care
NLK
Free Medical Journals
from 1996 to 1 year ago
Open Access Digital Library
from 1996-01-01
Medline Complete (EBSCOhost)
from 1999-01-01 to 1 year ago
- MeSH
- Amino Acyl-tRNA Synthetases immunology MeSH
- Autoantibodies * blood immunology MeSH
- Dermatomyositis * immunology complications MeSH
- Adult MeSH
- Exanthema etiology MeSH
- Lung Diseases, Interstitial immunology etiology MeSH
- Middle Aged MeSH
- Humans MeSH
- Myositis * immunology complications MeSH
- Neoplasms complications MeSH
- Registries * MeSH
- Aged MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Comparative Study MeSH
OBJECTIVES: To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. METHODS: Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). RESULTS: In total 1054 patients were included (DM, n = 405; ASyS, n = 649). In the ASyS cohort, 31% (n = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P < 0.001), whereas higher frequency of any of four DM-type rashes-heliotrope rash (n = 248, 61% vs n = 90, 44%), violaceous rash (n = 166, 41% vs n = 57, 9%), V-sign (n = 124, 31% vs n = 28, 4%), and shawl sign (n = 133, 33% vs n = 18, 3%)-differentiated DM from ASyS-DMskin (all P < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n = 67, 17%) compared with ASyS (n = 21, 3%) and ASyS-DMskin (n = 7, 3%) cohorts (both P < 0.001). CONCLUSION: DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management.
Center for Rheumatology and Spine Diseases Copenhagen University Hospital Copenhagen Denmark
Department of Immunology University of Debrecen Debrecen Hajdú Bihar Hungary
Department of Neurology Universitair Ziekenhuis Gent Ghent Belgium
Department of Neurology University Medical Center Göttingen Göttingen Germany
Department of Pharmacy and Pharmacology University of Bath Bath UK
Department of Rheumatology Alder Hey Children's NHS Foundation Trust Liverpool UK
Department of Rheumatology and Immunology Inselspital University Hospital Bern Bern Switzerland
Department of Rheumatology China Japan Friendship Hospital Beijing China
Department of Rheumatology Mayo Clinic Rochester MN USA
Department of Rheumatology Oslo University Hospital Oslo Norway
Department of Rheumatology Royal National Hospital for Rheumatic Diseases Bath UK
Division of Rheumatology Department of Medicine Karolinska Institutet Stockholm Sweden
Faculty of Science and Engineering Manchester Metropolitan University Manchester UK
Institute of Rheumatology and Department of Rheumatology Charles University Praha Czech Republic
Internal Medicine Department Hanoi Medical University Hanoi Vietnam
MRC ARUK Institute of Ageing and Chronic Disease University of Liverpool Liverpool UK
References provided by Crossref.org
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