학술논문

The EuroMyositis registry: an international collaborative tool to facilitate myositis research
Document Type
Academic Journal
Author
Lilleker, James BVencovsky, JiriWang, GuochunWedderburn, Lucy RDiederichsen, Louise PyndtSchmidt, JensOakley, PaulaBenveniste, OlivierDanieli, Maria GiovannaDanko, KatalinThuy, Nguyen Thi PhuongVazquez-Del Mercado, MonicaAndersson, HelenaDe Paepe, BoeldeBleecker, Jan LMaurer, BrittaMcCann, Liza JPipitone, NicoloMcHugh, NeilBetteridge, Zoe ENew, PaulCooper, Robert GOllier, William ELamb, Janine AKrogh, Niels SteenLundberg, Ingrid EChinoy, HectorD’hose, SophieTian, Xin Lu,xiaolanMann, HermanKryštufková, OlgaPleštilová, LenkaBarochová,katerina kubínová, Martin Klein,terezaGelardi, ChiaraPedini, VeronicaCardinaletti, PaoloJara, Luis JSaavedra, Miguel ACruz-reyes, Claudia VVera-lastra, OlgaAndrade-ortega, LiliaMedrano-ramírez, GabrielSatoh, MinoruSalazar-páramo, MarioGomez-bañuelos, EduardoAguilar-arreola, JorgeDurán-barragán, SergioNavarro-hernandez, Rosa ElenaPetri, Marcelo HMolberg, ØyvindDastmalchi, MaryamNotarnicola, AntonellaGheorghe, KarinaRönnelid, JohanLiden, MariaHanna, BalsamJalal, AwatHellström, HelenaMartineus, Jehns ChristianNgoc lan, Nguyen ThiPadyukov, LeonidNew, PaulPlatt, HazelRothwell, SimonAhmed, YasmeenArmstrong, RaymondBernstein, RobertBlack, CarolBowman, SimonBruce, IanButler, RobinCarty, JohnChattopadhyay, ChandraChelliah, EaswaradhasClarke, FionaDawes, PeterDenton, ChristopherDevlin, JosephEdwards, ChristopherEmery, PaulFordham, JohnFraser, AlexanderGaston, HillGordon, PatrickGriffiths, BridgetGunawardena, HarshaHall, FrancesHanna, MichaelHarrison, BeverleyHay, ElaineHilton-jones, DavidHorden, LesleyIsaacs, JohnIsenberg, DavidJones, AdrianKamath, SanjeetKennedy, ThomasKitas, GeorgeKlimiuk, PeterKnights, SallyLambert, JohnLanyon, PeterLaxminarayan, RamasharanLecky, BryanLuqmani, RaashidMachado, PedroMarks, JeffreyMartin, MichaelMcgonagle, DennisMchugh, NeilMckenna, FrancisMclaren, JohnMcmahon, MichaelMcrorie, EuanMerry, PeterMiles, SarahMiller, JamesNicholls, AnneNixon, JenniferOng, VoonOver, KatherinePackham, JohnPipitone, NicoloPlant, MichaelPountain, GillianPullar, ThomasRoberts, MarkSanders, PaulScott, DavidScott, DavidShadforth, MichaelSheeran, ThomasSrinivasan, ArulSwinson, DavidTeh, Lee-suanWebley, MichaelWilliams, BrianWiner, Jonathan
Source
Annals of the Rheumatic Diseases. Jan 01, 2018 77(1):30-39
Subject
Language
English
ISSN
0003-4967
Abstract
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.