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This included 28 patients with PM, IMNM and IBM that had the presence of a DM-specific rash recorded and 27 patients with DM where the presence of a DM-specific rash could not be confirmed.

The Registry categorises statin-related myotoxicity (SRM) cases using definitions suggested by Alfirevic (SRM1–6).29 We reclassified as IMNM any case with statin-associated IMNM (SRM6) or SRM occurring in association with 3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) autoantibodies.

We also reclassified as IMNM any case of PM that had anti-SRP or anti-HMGCR autoantibodies.

IBM cases met either the Medical Research Council, Griggs or European Neuromuscular Centre diagnostic criteria.19–21Those with suspected PM, DM or JDM, who did not fulfil these criteria were classified as ‘unspecified myositis’ and excluded from further analysis, unless they met the criteria for ASS (in which case they were analysed within the ASS group, see below) or had myositis overlapping with a CTD (in which case they were analysed with the CTD-overlap myositis group).

CTD-overlap myositis was defined as PM, DM, JDM or unspecified myositis coexisting with a CTD that met relevant diagnostic criteria.22–26 This report uses the term JDM for current adults with juvenile-onset (.27 This included retrospective reclassification of those with PM, DM, JDM or CTD-overlap myositis cases as having ASS if they possessed an antisynthetase autoantibody (anti-Jo1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-KS or anti-Zo autoantibodies), where results of these were available.

Immune-mediated necrotising myopathy (IMNM) and the antisynthetase syndrome (ASS) are also now recognised as distinct entities under the IIM umbrella.1 The importance of serotype in predicting clinical features and prognosis is becoming increasingly recognised.2Estimates of the prevalence of IIM vary widely from 0.55 to 17.50 per 1 00 000 people.3 The rarity and heterogeneity of IIM has hampered research efforts and impeded the delivery of large-scale interventional clinical trials.4 Consequently, the therapeutic evidence-base in IIM is remarkably limited.5 6 Recently, several IIM research databases and registries have been created to pool resources and expertise, facilitating completion of several international IIM research studies.7–11We describe the data held within Euro Myositis, the largest IIM disease registry, highlighting the differing clinical characteristics of each IIM diagnostic subtype and analyse associations with extramuscular involvement, malignancy, environmental exposures and disease severity.

Anonymised downloads from those agreeing to participate (Belgium, China, Czech Republic, Hungary, Italy, Mexico, Norway, Sweden, Switzerland, the UK and Vietnam) were obtained on 15 August 2016, including 92% (3487/3790) of all cases in the Registry.

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