欧洲肌炎注册库：一个促进肌炎研究的国际协作工具。
The EuroMyositis registry: an international collaborative tool to facilitate myositis research.
作者信息
Lilleker James B, Vencovsky Jiri, Wang Guochun, Wedderburn Lucy R, Diederichsen Louise Pyndt, Schmidt Jens, Oakley Paula, Benveniste Olivier, Danieli Maria Giovanna, Danko Katalin, Thuy Nguyen Thi Phuong, Vazquez-Del Mercado Monica, Andersson Helena, De Paepe Boel, deBleecker Jan L, Maurer Britta, McCann Liza J, Pipitone Nicolo, McHugh Neil, Betteridge Zoe E, New Paul, Cooper Robert G, Ollier William E, Lamb Janine A, Krogh Niels Steen, Lundberg Ingrid E, Chinoy Hector
机构信息
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.
Greater Manchester Neurosciences Centre, Salford Royal NHS Foundation Trust, Salford, UK.
出版信息
Ann Rheum Dis. 2018 Jan;77(1):30-39. doi: 10.1136/annrheumdis-2017-211868. Epub 2017 Aug 30.
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.
目的
欧洲肌炎注册登记处促进了特发性炎性肌病（IIM）研究群体之间的合作。本首份报告分析了注册登记处的汇总数据。
方法
对来自11个国家的IIM病例进行横断面分析。研究临床亚型、肌肉外受累情况、环境暴露因素和药物之间的关联。
结果
在3067例IIM病例中，69%为女性。最常见的IIM亚型是皮肌炎（DM）（31%）。结缔组织病重叠综合征病例中吸烟更为常见（45%，比值比1.44，95%置信区间1.09至1.90，p = 0.012）。吸烟与间质性肺病（ILD）相关（比值比1.32，95%置信区间1.06至1.65，p = 0.013）、吞咽困难（比值比1.43，95%置信区间1.16至1.77，p = 0.001）、既往有恶性肿瘤（比值比1.78，95%置信区间1.36至2.33，p<0.001）和心脏受累（比值比2.40，95%置信区间1.60至3.60，p<0.001）。39%的病例出现吞咽困难，9%的病例出现心脏受累；二者任何一种情况均与较高的健康评估问卷（HAQ）评分相关（校正比值比1.79，95%置信区间1.43至2.23，p<0.001）。包涵体肌炎病例的HAQ评分也较高（校正比值比3.85，95%置信区间2.52至5.90，p<0.001）。13%的病例有恶性肿瘤（既往有），最常见于DM（20%，比值比2.06，95%置信区间1.65至2.57，p<0.001）。30%的病例出现ILD，最常见于抗合成酶综合征（71%，比值比10.7，95%置信区间8.6至13.4，p<0.001）。成人起病和青少年起病的DM病例皮疹特征不同（“V”征：DM为56%，青少年DM为16%，比值比0.16，95%置信区间0.07至0.36，p<0.001）。98%的病例使用糖皮质激素，71%使用甲氨蝶呤，51%使用硫唑嘌呤。
结论
这个大型多中心队列证明了肌肉外受累在IIM患者中的重要性、其与吸烟的关联及其对疾病严重程度的影响。我们的研究结果强调IIM是一种多系统炎性疾病，并将有助于为患者的预后和临床管理提供信息。
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