University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany.
Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
Arthritis Rheumatol. 2019 Sep;71(9):1400-1412. doi: 10.1002/art.40930. Epub 2019 Aug 6.
To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR).
This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects.
The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.
These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research.
由欧洲抗风湿病联盟（EULAR）和美国风湿病学会（ACR）共同制定新的系统性红斑狼疮（SLE）分类标准。
这项国际倡议分为四个阶段。1）通过系统评价和文献荟萃分析以及国际德尔菲专家咨询、早期患者队列和患者调查，评估抗核抗体（ANA）作为纳入标准的作用，并生成分类标准。2）通过德尔菲法和名义群体技术进行标准简化。3）根据标准性能和多准则决策分析的结果，定义和加权标准。4）在 1001 例新推导队列中进一步完善权重和阈值评分，并与 1270 例新验证队列中的以前标准进行验证比较。
2019 年 EULAR/ACR SLE 分类标准包括至少一次阳性 ANA 作为强制性纳入标准；随后是附加加权标准，分为 7 个临床（全身、血液、神经精神、黏膜皮肤、浆膜、肌肉骨骼、肾脏）和 3 个免疫学（抗磷脂抗体、补体蛋白、SLE 特异性抗体）域，权重为 2 到 10。累积≥10 分的患者可被分类。在验证队列中，新的标准具有 96.1%的敏感性和 93.4%的特异性，而 ACR 1997 标准的敏感性为 82.8%，特异性为 93.4%，SLE 国际协作临床 2012 标准的敏感性为 96.7%，特异性为 83.7%。
这些新的分类标准是使用多学科和国际投入的严格方法制定的，具有优异的敏感性和特异性。使用 ANA 纳入标准、分层聚类和加权标准反映了目前对 SLE 的认识，为 SLE 研究提供了更好的基础。
