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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.
Original language | English |
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Pages (from-to) | 30-39 |
Journal | Annals of the rheumatic diseases |
Volume | 77 |
Issue number | 1 |
Early online date | 30 Aug 2017 |
DOIs | |
Publication status | Published - 2018 |
Keywords
- DISEASE REGISTRIES
- MYOSITIS
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MMRG: Manchester Myositis Research Group
Chinoy, H. (PI), Lamb, J. (PI), Ollier, W. (PI), Rothwell, S. (CoI), Lilleker, J. (CoI), Oldroyd, A. (PGR student), Snedden, A. (PGR student), Platt, H. (Support team) & New, P. (Support team)
1/01/10 → …
Project: Research
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