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

Abstract

AimsThe EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data.MethodsCross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated.ResultsOf 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%.ConclusionThis 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.

Overlap myositis, a distinct entity beyond primary inflammatory myositis: A retrospective analysis of a large cohort from the REMICAM registry

“…The description of OM patients in the literature is scant, with short series in most cases. Furthermore, wide variations have been reported, depending on the inclusion criteria and definition of OM, time of follow‐up or ethnic differences between populations . Large cohorts show that 12%‐67% of patients with IIM are classified as OM, although the majority of studies include low numbers of patients with OM .…”

Assessment of swallowing function with oro‐pharyngeal‐esophageal scintigraphy in patients with idiopathic inflammatory myopathies

“…Dysphagia still represents one of the most common extra‐muscular involvements; it is present in up to 84% of IIM patients and is associated to severe reduction of the quality of life, particularly in patients with specific myositis‐specific autoantibodies (eg, Mi2), IBM, and cancer‐associated myositis …”

Response to: ‘Antisynthetase syndrome or what else? Different perspectives indicate the need for new classification criteria’ by Cavagnaet al

“…We thank Cavagna et al 1 for their thoughtful analysis relating to our recent publication 2. Several important points are raised, many of which we also referred to.…”