The EuroMyositis registry: an international collaborative tool to facilitate myositis research



Lilleker, James B, Vencovsky, Jiri, Wang, Guochun, Wedderburn, Lucy R, Diederichsen, Louise Pyndt, Schmidt, Jens, Oakley, Paula, Benveniste, Olivier, Danieli, Maria Giovanna, Danko, Katalin
et al (show 17 more authors) (2018) The EuroMyositis registry: an international collaborative tool to facilitate myositis research. ANNALS OF THE RHEUMATIC DISEASES, 77 (1). pp. 30-39.

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Abstract

<h4>Aims</h4>The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data.<h4>Methods</h4>Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated.<h4>Results</h4>Of 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).Dysphagia 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% DM 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%.<h4>Conclusion</h4>This 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.

Item Type: Article
Uncontrolled Keywords: all EuroMyositis contributors, Humans, Myositis, Prognosis, Severity of Illness Index, Registries, Cohort Studies, Cross-Sectional Studies, Smoking, Health Status, Biomedical Research, International Cooperation, Female, Male, Surveys and Questionnaires
Depositing User: Symplectic Admin
Date Deposited: 25 Sep 2017 15:49
Last Modified: 19 Jan 2023 06:53
DOI: 10.1136/annrheumdis-2017-211868
Related URLs:
URI: https://livrepository.liverpool.ac.uk/id/eprint/3009624