TY - JOUR
T1 - The EuroMyositis registry
T2 - an international collaborative tool to facilitate myositis research
AU - Lilleker, James B
AU - Vencovsky, Jiri
AU - Wang, Guochun
AU - Wedderburn, Lucy R
AU - Diederichsen, Louise Pyndt
AU - Schmidt, Jens
AU - Oakley, Paula
AU - Benveniste, Olivier
AU - Danieli, Maria Giovanna
AU - Danko, Katalin
AU - Thuy, Nguyen Thi Phuong
AU - Vazquez-Del Mercado, Monica
AU - Andersson, Helena
AU - De Paepe, Boel
AU - deBleecker, Jan L
AU - Maurer, Britta
AU - McCann, Liza J
AU - Pipitone, Nicolo
AU - McHugh, Neil
AU - Betteridge, Zoe E
AU - New, Paul
AU - Cooper, Robert G
AU - Ollier, William E
AU - Lamb, Janine A
AU - Krogh, Niels Steen
AU - Lundberg, Ingrid E
AU - Chinoy, Hector
AU - all EuroMyositis contributors
N1 - © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
PY - 2017/12/11
Y1 - 2017/12/11
N2 - Aims T he EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. Methods Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. Results O f 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%). Smoking was more frequent in connective tissue disease overlap cases (45%, OR 1.44, 95% CI 1.09 to 1.90, p=0.012). Smoking was associated with interstitial lung disease (ILD) (OR 1.32, 95% CI 1.06 to 1.65, p=0.013), dysphagia (OR 1.43, 95% CI 1.16 to 1.77, p=0.001), malignancy ever (OR 1.78, 95% CI 1.36 to 2.33, p<0.001) and cardiac involvement (OR 2.40, 95% CI 1.60 to 3.60, p<0.001). D ysphagia occurred in 39% and cardiac involvement in 9%; either occurrence was associated with higher Health Assessment Questionnaire (HAQ) scores (adjusted OR 1.79, 95% CI 1.43 to 2.23, p<0.001). HAQ scores were also higher in inclusion body myositis cases (adjusted OR 3.85, 95% CI 2.52 to 5.90, p<0.001). Malignancy (ever) occurred in 13%, most commonly in DM (20%, OR 2.06, 95% CI 1.65 to 2.57, p<0.001). ILD occurred in 30%, most frequently in antisynthetase syndrome (71%, OR 10.7, 95% CI 8.6 to 13.4, p<0.001). Rash characteristics differed between adult-onset and juvenile-onset DM cases ('V' sign: 56% D M vs 16% juvenile-DM, OR 0.16, 95% CI 0.07 to 0.36, p<0.001). Glucocorticoids were used in 98% of cases, methotrexate in 71% and azathioprine in 51%. Conclusion T his large multicentre cohort demonstrates the importance of extramuscular involvement in patients with IIM, its association with smoking and its influence on disease severity. Our findings emphasise that IIM is a multisystem inflammatory disease and will help inform prognosis and clinical management of patients.
AB - Aims T he EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. Methods Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. Results O f 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%). Smoking was more frequent in connective tissue disease overlap cases (45%, OR 1.44, 95% CI 1.09 to 1.90, p=0.012). Smoking was associated with interstitial lung disease (ILD) (OR 1.32, 95% CI 1.06 to 1.65, p=0.013), dysphagia (OR 1.43, 95% CI 1.16 to 1.77, p=0.001), malignancy ever (OR 1.78, 95% CI 1.36 to 2.33, p<0.001) and cardiac involvement (OR 2.40, 95% CI 1.60 to 3.60, p<0.001). D ysphagia occurred in 39% and cardiac involvement in 9%; either occurrence was associated with higher Health Assessment Questionnaire (HAQ) scores (adjusted OR 1.79, 95% CI 1.43 to 2.23, p<0.001). HAQ scores were also higher in inclusion body myositis cases (adjusted OR 3.85, 95% CI 2.52 to 5.90, p<0.001). Malignancy (ever) occurred in 13%, most commonly in DM (20%, OR 2.06, 95% CI 1.65 to 2.57, p<0.001). ILD occurred in 30%, most frequently in antisynthetase syndrome (71%, OR 10.7, 95% CI 8.6 to 13.4, p<0.001). Rash characteristics differed between adult-onset and juvenile-onset DM cases ('V' sign: 56% D M vs 16% juvenile-DM, OR 0.16, 95% CI 0.07 to 0.36, p<0.001). Glucocorticoids were used in 98% of cases, methotrexate in 71% and azathioprine in 51%. Conclusion T his large multicentre cohort demonstrates the importance of extramuscular involvement in patients with IIM, its association with smoking and its influence on disease severity. Our findings emphasise that IIM is a multisystem inflammatory disease and will help inform prognosis and clinical management of patients.
KW - Biomedical Research/methods
KW - Cohort Studies
KW - Cross-Sectional Studies
KW - Female
KW - Health Status
KW - Humans
KW - International Cooperation
KW - Male
KW - Myositis/epidemiology
KW - Prognosis
KW - Registries/statistics & numerical data
KW - Severity of Illness Index
KW - Smoking/adverse effects
KW - Surveys and Questionnaires
UR - http://www.scopus.com/inward/record.url?scp=85038218055&partnerID=8YFLogxK
U2 - 10.1136/annrheumdis-2017-211868
DO - 10.1136/annrheumdis-2017-211868
M3 - Article
C2 - 28855174
SN - 0003-4967
VL - 77
SP - 30
EP - 39
JO - Annals of the Rheumatic Diseases
JF - Annals of the Rheumatic Diseases
IS - 1
ER -
