Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa Italy and Department of Medical Biotechnology, University of Siena, Siena, Italy.
Division of Rheumatology, Department of Medicine, Karolinska Institutet, and Karolinska University Hospital, Solna, Stockholm SE-171 76, Sweden.
Semin Arthritis Rheum. 2020 Jun;50(3):492-497. doi: 10.1016/j.semarthrit.2019.12.001. Epub 2019 Dec 28.
In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) published new classification criteria for idiopathic inflammatory myopathies (IIM).
To [1] assess the performance of the EULAR/ACR criteria in a monocentric cohort of consecutive patients with IIM, compare them with the Bohan and Peter (BP) criteria, and with the physician's diagnosis; and [2] evaluate the effect of including the presence of interstitial lung disease (ILD) as variable in the criteria.
439 consecutive patients with a diagnosis of IIM followed at the Rheumatology Clinic, Karolinska University Hospital, Sweden were enrolled. The patients were diagnosed as IIM and subclassified by expert physicians. Clinical, laboratory, serological and histopathological data were collected from existing databases (Euromyositis registry and Swedish Rheumatology quality registry) and clinical charts of the patients. The sensitivity of the EULAR/ACR and the BP criteria was calculated.
The EULAR/ACR criteria had a higher sensitivity (87.7%) compared to the BP criteria (80.4%). The concordance between the two sets of criteria was low (k = 0.253 p<0.001). The EULAR/ACR criteria showed a very high specificity (>98%) for the major IIM subgroups polymyositis, dermatomyositis, and inclusion body myositis. The sensitivity was variable and was high in inclusion body myositis (98%), dermatomyositis (90%) and lower in polymyositis (73%). When including ILD in the variables of the criteria, six more patients were classified as IIM cases (1.3%).
The EULAR/ACR criteria for IIM are applicable with high sensitivity and specificity using data available from existing databases and clinical charts and represent a major step forward from the previous criteria for IIM and its subgroups. Their application will improve the quality of clinical trials and research studies with IIM patients.
2017 年，欧洲抗风湿病联盟（EULAR）和美国风湿病学会（ACR）发布了特发性炎性肌病（IIM）的新分类标准。
[1]评估 EULAR/ACR 标准在单中心连续 IIM 患者队列中的表现，将其与 Bohan 和 Peter（BP）标准进行比较，并与医生的诊断进行比较；[2]评估将间质性肺病（ILD）的存在作为标准中的变量的影响。
在瑞典卡罗林斯卡大学医院风湿病科就诊的 439 例 IIM 患者连续入选。患者由专家医生诊断为 IIM 并进行亚分类。临床、实验室、血清学和组织病理学数据来自现有的数据库（Euromyositis 登记处和瑞典风湿病质量登记处）和患者的临床病历。计算 EULAR/ACR 和 BP 标准的敏感性。
EULAR/ACR 标准的敏感性（87.7%）高于 BP 标准（80.4%）。两种标准之间的一致性较低（k=0.253，p<0.001）。EULAR/ACR 标准对主要 IIM 亚组多发性肌炎、皮肌炎和包涵体肌炎具有很高的特异性（>98%）。敏感性是可变的，包涵体肌炎（98%）、皮肌炎（90%）较高，多发性肌炎（73%）较低。当将ILD 纳入标准的变量时，又有 6 例患者被归类为 IIM 病例（1.3%）。
EULAR/ACR 标准适用于使用现有数据库和临床病历中可用的数据，具有较高的敏感性和特异性，代表了 IIM 及其亚组的先前标准的重大进步。它们的应用将提高 IIM 患者临床试验和研究的质量。
