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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 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.
Keywords
IDIOPATHIC INFLAMMATORY MYOPATHIES, CLASSIFICATION, DERMATOMYOSITIS, AUTOANTIBODIES, ADULT, POLYMYOSITIS, CRITERIA, DISEASE, ANTIBODIES, DIAGNOSIS

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Chicago
Lilleker, James B, Jiri Vencovsky, Guochun Wang, Lucy R Wedderburn, Louise Pyndt Diederichsen, Jens Schmidt, Paula Oakley, et al. 2018. “The EuroMyositis Registry : an International Collaborative Tool to Facilitate Myositis Research.” Annals of the Rheumatic Diseases 77 (1): 30–39.
APA
Lilleker, J. B., Vencovsky, J., Wang, G., Wedderburn, L. R., Diederichsen, L. P., Schmidt, J., Oakley, P., et al. (2018). The EuroMyositis registry : an international collaborative tool to facilitate myositis research. ANNALS OF THE RHEUMATIC DISEASES, 77(1), 30–39.
Vancouver
1.
Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J, et al. The EuroMyositis registry : an international collaborative tool to facilitate myositis research. ANNALS OF THE RHEUMATIC DISEASES. 2018;77(1):30–9.
MLA
Lilleker, James B, Jiri Vencovsky, Guochun Wang, et al. “The EuroMyositis Registry : an International Collaborative Tool to Facilitate Myositis Research.” ANNALS OF THE RHEUMATIC DISEASES 77.1 (2018): 30–39. Print.
@article{8529960,
  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 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{\textlangle}0.001) and cardiac involvement (OR 2.40, 95\% CI 1.60 to 3.60, p{\textlangle}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{\textlangle}0.001). HAQ scores were also higher in inclusion body myositis cases (adjusted OR 3.85, 95\% CI 2.52 to 5.90, p{\textlangle}0.001). Malignancy (ever) occurred in 13\%, most commonly in DM (20\%, OR 2.06, 95\% CI 1.65 to 2.57, p{\textlangle}0.001). ILD occurred in 30\%, most frequently in antisynthetase syndrome (71\%, OR 10.7, 95\% CI 8.6 to 13.4, p{\textlangle}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{\textlangle}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.},
  author       = {Lilleker, James B and Vencovsky, Jiri and Wang, Guochun and Wedderburn, Lucy R and Diederichsen, Louise Pyndt and Schmidt, Jens and Oakley, Paula and Benveniste, Olivier and Danieli, Maria Giovanna and Danko, Katalin and Thuy, Nguyen Thi Phuong and Vazquez-Del Mercado, Monica and Andersson, Helena and De Paepe, Boel and De Bleecker, Jan and Maurer, Britta and McCann, Liza J and Pipitone, Nicolo and McHugh, Neil and Betteridge, Zoe E and New, Paul and Cooper, Robert G and Ollier, William E and Lamb, Janine A and Steen Krogh, Niels and Lundberg, Ingrid E and Chinoy, Hector},
  issn         = {0003-4967},
  journal      = {ANNALS OF THE RHEUMATIC DISEASES},
  keyword      = {IDIOPATHIC INFLAMMATORY MYOPATHIES,CLASSIFICATION,DERMATOMYOSITIS,AUTOANTIBODIES,ADULT,POLYMYOSITIS,CRITERIA,DISEASE,ANTIBODIES,DIAGNOSIS},
  language     = {eng},
  number       = {1},
  pages        = {30--39},
  title        = {The EuroMyositis registry : an international collaborative tool to facilitate myositis research},
  url          = {http://dx.doi.org/10.1136/annrheumdis-2017-211868},
  volume       = {77},
  year         = {2018},
}

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