Lookup NU author(s): Dr Bridget Griffiths,
Professor John Isaacs
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
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.
Author(s): Lilleker JB, Vencovsky J, Wang G, Wedderburn LR, Diederichsen LP, Schmidt J, Oakley P, Benveniste O, Danieli MG, Danko K, Thuy NTP, Vazquez-Del Mercado M, Andersson H, De Paepe B, DeBleecker JL, Maurer B, McCann LJ, Pipitone N, McHugh N, Betteridge ZE, New P, Cooper RG, Ollier WE, Lamb JA, Krogh NS, Lundberg IE, Chinoy H, D'Hose S, Lu X, Tian X, Mann H, Krystufkova O, Plestilova L, Klein M, Barochova T, Kubinova K, Gelardi C, Pedini V, Cardinaletti P, Jara LJ, Saavedra MA, Cruz-Reyes CV, Vera-Lastra O, Andrade-Ortega L, Medrano-Ramirez G, Satoh M, Salazar-Paramo M, Gomez-Banuelos E, Aguilar-Arreola J, Duran-Barragan S, Navarro-Hernandez RE, Petri MH, Molberg O, Dastmalchi M, Notarnicola A, Gheorghe K, Ronnelid J, Liden M, Hanna B, Jalal A, Hellstrom H, Martineus JC, Lan NTN, Padyukov L, Platt H, Rothwell S, Ahmed Y, Armstrong R, Bernstein R, Black C, Bowman S, Bruce I, Butler R, Carty J, Chattopadhyay C, Chelliah E, Clarke F, Dawes P, Denton C, Devlin J, Edwards C, Emery P, Fordham J, Fraser A, Gaston H, Gordon P, Griffiths B, Gunawardena H, Hall F, Hanna M, Harrison B, Hay E, Hilton-Jones D, Horden L, Isaacs J, Isenberg D, Jones A, Kamath S, Kennedy T, Kitas G, Klimiuk P, Knights S, Lambert J, Lanyon P, Laxminarayan R, Lecky B, Luqmani R, Machado P, Marks J, Martin M, McGonagle D, McKenna F, McLaren J, McMahon M, McRorie E, Merry P, Miles S, Miller J, Nicholls A, Nixon J, Ong V, Over K, Packham J, Plant M, Pountain G, Pullar T, Roberts M, Sanders P, Scott D, Shadforth M, Sheeran T, Srinivasan A, Swinson D, The L-S, Webley M, Williams B, Winer J
Publication type: Article
Publication status: Published
Journal: Annals of the Rheumatic Diseases
Online publication date: 30/08/2017
Acceptance date: 01/08/2017
Date deposited: 14/06/2018
ISSN (print): 0003-4967
ISSN (electronic): 1468-2060
Publisher: BMJ Publishing Group
PubMed id: 28855174
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