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.

Clinica Medica Dipartimento di Scienze Cliniche e Molecolari Università Politecnica delle Marche and Ospedali Riuniti Ancona Italy

Département de Médecine Interne et Immunologie Clinique Hôpital Pitié Salpêtrière AP HP UPMC Paris France

Department of Neurology Ghent University Hospital Ghent Belgium

Department of Neurology University Medical Center Göttingen Göttingen Germany

Department of Pharmacy and Pharmacology University of Bath Bath UK

Department of Rheumatology Alder Hey Children's NHS Foundation Trust Liverpool UK

Department of Rheumatology Arcispedale S Maria Nuova IRCCS of Reggio Emilia Reggio Emilia Italy

Department of Rheumatology Bach Mai Hospital Bach Mai Hospital Hanoi Medical University Hanoi Viet Nam

Department of Rheumatology China Japan Friendship Hospital Beijing China

Department of Rheumatology Odense University Hospital Odense Denmark

Department of Rheumatology Oslo University Hospital Oslo Norway

Department of Rheumatology Salford Royal NHS Foundation Trust Manchester Academic Health Science Centre Salford UK

Department of Rheumatology University Hospital Zurich Zurich Switzerland

División de Medicina Interna Universidad de Guadalajara Guadalajara Jalisco Mexico

Division of Immunology University of Debrecen Debrecen Hungary

Division of Musculoskeletal and Dermatological Sciences Centre for Musculoskeletal Research School of Biological Sciences Faculty of Biology Medicine and Health Manchester Academic Health Science Centre The University of Manchester Manchester UK

Division of Population Health Health Services Research and Primary Care School of Health Sciences Faculty of Biology Medicine and Health Manchester Academic Health Science Centre The University of Manchester Manchester UK

Greater Manchester Neurosciences Centre Salford Royal NHS Foundation Trust Salford UK

Institute of Rheumatology Prague Czech Republic

MRC ARUK Institute of Ageing and Chronic Disease University of Liverpool Liverpool UK

Myositis UK Southampton UK

Royal National Hospital for Rheumatic Diseases Bath Bath and North East Somer UK

The National Institute for Health Research Manchester Musculoskeletal Biomedical Research Unit Central Manchester University Hospitals NHS Foundation Trust Manchester Academic Health Science Centre The University of Manchester Manchester UK

Unit of Rheumatology Department of Medicine Karolinska University Hospital Solna Karolinska Institutet Stockholm Sweden

University College London GOS Institute of Child Health and NIHR GOSH Biomedical Research Centre Great Ormond Street Hospital for Children NHS Trust London UK

Zitelab Aps Copenhagen Denmark

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