2013年系统性硬化症分类标准：美国风湿病学会/欧洲抗风湿病联盟合作倡议
2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.
作者信息
van den Hoogen Frank, Khanna Dinesh, Fransen Jaap, Johnson Sindhu R, Baron Murray, Tyndall Alan, Matucci-Cerinic Marco, Naden Raymond P, Medsger Thomas A, Carreira Patricia E, Riemekasten Gabriela, Clements Philip J, Denton Christopher P, Distler Oliver, Allanore Yannick, Furst Daniel E, Gabrielli Armando, Mayes Maureen D, van Laar Jacob M, Seibold James R, Czirjak Laszlo, Steen Virginia D, Inanc Murat, Kowal-Bielecka Otylia, Müller-Ladner Ulf, Valentini Gabriele, Veale Douglas J, Vonk Madelon C, Walker Ulrich A, Chung Lorinda, Collier David H, Csuka Mary Ellen, Fessler Barri J, Guiducci Serena, Herrick Ariane, Hsu Vivien M, Jimenez Sergio, Kahaleh Bashar, Merkel Peter A, Sierakowski Stanislav, Silver Richard M, Simms Robert W, Varga John, Pope Janet E
机构信息
St. Maartenskliniek and Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
出版信息
Arthritis Rheum. 2013 Nov;65(11):2737-47. doi: 10.1002/art.38098. Epub 2013 Oct 3.
OBJECTIVE
The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc.
METHODS
Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc.
RESULTS
It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc.
CONCLUSION
The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.
目的
1980年美国风湿病学会（ACR）系统性硬化症（SSc）分类标准对早期SSc和局限性皮肤型SSc缺乏敏感性。由ACR和欧洲抗风湿病联盟（EULAR）联合委员会开展的本研究旨在制定SSc的新分类标准。
方法
采用共识方法，将23个候选项目安排在一个多标准加分系统中，并设定一个阈值以将病例分类为SSc。通过对项目进行聚类和简化权重来简化分类系统。该系统通过以下方式进行测试：1）确定SSc病例和硬皮病样疾病对照中的特异性和敏感性；2）根据一组专家对一组有或无SSc病例的综合意见进行验证。
结果
确定手指皮肤增厚延伸至掌指关节近端足以将患者分类为患有SSc；如果不存在这种情况，则适用7个加分项目，每个项目权重不同：手指皮肤增厚、指尖病变、毛细血管扩张、甲襞毛细血管异常、间质性肺病或肺动脉高压、雷诺现象以及与SSc相关的自身抗体。新分类标准在验证样本中的敏感性和特异性分别为0.91和0.92，而1980年ACR分类标准的敏感性和特异性分别为0.75和0.72。所有选定病例均根据基于共识的专家意见进行分类。所有根据1980年ACR标准分类为SSc的病例，按照新标准也被分类为SSc，并且现在有几个额外的病例被认为是SSc。
结论
ACR/EULAR的SSc分类标准比1980年ACR的SSc标准表现更好，应该能使更多患者被正确分类为患有该病。
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