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

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Abstract

Aims:The 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: Of 3,067 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%, odds ratio [OR] 1.44, 95% confidence interval 1.09-1.90, p=0.012). Smoking was associated with ILD (1.32, 1.06-1.65, p=0.013), dysphagia (1.43, 1.16-1.77, p=0.001), malignancy ever (1.78, 1.36-2.33, p<0.001) and cardiac involvement (2.40, 1.60-3.60, 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, 1.43-2.23, p<0.001). HAQ scores were also higher in inclusion body myositis cases (adjusted OR 3.85, 2.52-5.90, p<0.001). Malignancy (ever) occurred in 13%, most commonly in DM (20%, 2.06, 1.65-2.57, p<0.001).Interstitial lung disease (ILD) occurred in 30%, most frequently in anti-synthetase syndrome (71%, 10.7, 8.6-13.4, p<0.001). Rash characteristics differed between adult- and juvenile-onset DM cases (“V” sign: 56% DM versus 16% juvenile-DM, 0.16, 0.07-0.36, p<0.001). Glucocorticoids were used in 98% of cases, methotrexate in 71%, and azathioprine in 51%.Conclusion: 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 multi-system inflammatory disease and will help inform prognosis and clinical management of patients.

Bibliographical metadata

Original languageEnglish
Pages (from-to)30-39
JournalAnnals of the rheumatic diseases
Volume77
Issue number1
Early online date30 Aug 2017
DOIs
Publication statusPublished - 2018

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