Journal
ANNALS OF THE RHEUMATIC DISEASES
Volume 77, Issue 1, Pages 30-39Publisher
BMJ PUBLISHING GROUP
DOI: 10.1136/annrheumdis-2017-211868
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Funding
- Association Francaise Contre Les Myopathies (AFM)
- European Union Sixth Framework Programme (project AutoCure) [LSH-018661]
- European Science Foundation (ESF)
- Swedish Research Council [K2014-52X-14045-14-3]
- Stockholm County Council
- Karolinska Institutet
- Myositis UK
- Medical Research Council (MRC) UK [MR/N003322/1]
- Arthritis Research UK [18474]
- Project for Conceptual Development of Research Organization from Ministry of Health in the Czech Republic [00023728]
- Medical Research Council [MR/N003322/1, G0901461, MR/P020941/1, G0900753, G0100594, G0600237, MR/K002279/1] Funding Source: researchfish
- National Institute for Health Research [NF-SI-0617-10075, NF-SI-0513-10139, NF-SI-0512-10105, NF-SI-0508-10299, RC-PG-0407-10054, CL-2006-06-010] Funding Source: researchfish
- Versus Arthritis [18474, 18475, 20639] Funding Source: researchfish
- MRC [G0901461, G0900753, G0600237, MR/P020941/1, G0100594, MR/N003322/1, MR/K002279/1] Funding Source: UKRI
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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 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%. 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 multisystem inflammatory disease and will help inform prognosis and clinical management of patients.
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