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

James B Lilleker, Jiri Vencovsky, Guochun Wang, Lucy R Wedderburn, Louise Pyndt Diederichsen, Jens Schmidt, Paula Oakley, Olivier Benveniste, Maria Giovanna Danieli, Katalin Danko, Nguyen Thi Phuong Thuy, Monica Vazquez-Del Mercado, Helena Andersson, Boel De Paepe, Jan L deBleecker, Britta Maurer, Liza J McCann, Nicolo Pipitone, Neil McHugh, Zoe E BetteridgePaul New, Robert G Cooper, William E Ollier, Janine A Lamb, Niels Steen Krogh, Ingrid E Lundberg, Hector Chinoy, all EuroMyositis contributors

Research output: Contribution to journalArticle

51 Citations (Scopus)

Abstract

Aims T he 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 O f 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). D ysphagia 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% D M 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 T his 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.

Original languageEnglish
Pages (from-to)30-39
Number of pages10
JournalAnnals of the Rheumatic Diseases
Volume77
Issue number1
Early online date30 Aug 2017
DOIs
Publication statusPublished - 11 Dec 2017

Keywords

  • Biomedical Research/methods
  • Cohort Studies
  • Cross-Sectional Studies
  • Female
  • Health Status
  • Humans
  • International Cooperation
  • Male
  • Myositis/epidemiology
  • Prognosis
  • Registries/statistics & numerical data
  • Severity of Illness Index
  • Smoking/adverse effects
  • Surveys and Questionnaires

Fingerprint Dive into the research topics of 'The EuroMyositis registry: an international collaborative tool to facilitate myositis research'. Together they form a unique fingerprint.

  • Cite this

    Lilleker, J. B., Vencovsky, J., Wang, G., Wedderburn, L. R., Diederichsen, L. P., Schmidt, J., Oakley, P., Benveniste, O., Danieli, M. G., Danko, K., Thuy, N. T. P., Vazquez-Del Mercado, M., Andersson, H., De Paepe, B., deBleecker, J. L., Maurer, B., McCann, L. J., Pipitone, N., McHugh, N., ... all EuroMyositis contributors (2017). The EuroMyositis registry: an international collaborative tool to facilitate myositis research. Annals of the Rheumatic Diseases, 77(1), 30-39. https://doi.org/10.1136/annrheumdis-2017-211868