Department of Rheumatology, Clinical Immunology and Allergy, University of Crete School of Medicine, Iraklio, Greece.
4th Department of Medicine, 'Attikon' University Hospital, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
Ann Rheum Dis. 2020 Feb;79(2):232-241. doi: 10.1136/annrheumdis-2019-216155. Epub 2019 Nov 8.
Classification criteria are biased towards classifying long-standing disease. We compared the European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)-2019, Systemic Lupus International Collaborating Clinics (SLICC)-2012 and ACR-1997 criteria in an early (median 48 months) systemic lupus erythematosus (SLE) cohort.
Patients diagnosed with SLE (n=690) or control diseases (n=401). Sensitivity, specificity of the criteria and time-to-classification were calculated. Modified classification algorithms were derived from a random 80% and validated in the remaining 20% of the dataset running multiple iterations.
At last assessment, sensitivities of ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria were 85.7%, 91.3% and 88.6%, with specificities 93.0%, 93.8% and 97.3%, respectively. Both SLICC and EULAR/ACR enabled earlier classification. Only 76.7% of patients with SLE met all three criteria suggesting non-overlapping groups. Notably, unclassified patients had high prevalence of British Isles Lupus Assessment Group moderate/severe manifestations (43.3%-60%) and SLICC/ACR organ damage (30%-50%). At diagnosis, criteria missed 25.6%-30.5% of patients. Modification of EULAR/ACR and SLICC algorithms to include hypocomplementaemia and/or positive anti-phospholipid antibodies as alternative entry criterion, and/or allow classification with fewer clinical criteria from multiple organs, increased their sensitivity at diagnosis (median 82.0% and 86.2%) and overall (93.7% and 97.1%) with modest decreases in specificity. Importantly, patients who were still missed by the modified criteria had lower incidence of major organ involvement, use of immunosuppressive/biological therapies and organ damage.
The SLICC and EULAR/ACR are more sensitive than the ACR and the EULAR/ACR criteria have superior specificity in early SLE, although patients with significant disease can be missed. Combination and/or modification of the classification algorithms may enhance their sensitivity, allowing earlier classification and treatment of more patients with high disease burden.
分类标准偏向于对长期疾病进行分类。我们比较了欧洲抗风湿病联盟（EULAR）/美国风湿病学会（ACR）-2019、系统性红斑狼疮国际合作临床（SLICC）-2012 和 ACR-1997 标准在早期（中位数 48 个月）系统性红斑狼疮（SLE）队列中的表现。
患者被诊断为 SLE（n=690）或对照疾病（n=401）。计算了标准的敏感性、特异性和分类时间。从随机的 80%数据中得出修改后的分类算法，并在剩余的 20%数据中进行多次迭代验证。
在最后一次评估中，ACR-1997、SLICC-2012 和 EULAR/ACR-2019 标准的敏感性分别为 85.7%、91.3%和 88.6%，特异性分别为 93.0%、93.8%和 97.3%。SLICC 和 EULAR/ACR 都能更早地进行分类。只有 76.7%的 SLE 患者符合所有三个标准，这表明它们是不重叠的组。值得注意的是，未分类的患者有较高的不列颠群岛狼疮评估组中度/严重表现（43.3%-60%）和 SLICC/ACR 器官损害（30%-50%）。在诊断时，标准漏掉了 25.6%-30.5%的患者。修改 EULAR/ACR 和 SLICC 算法，将低补体血症和/或抗磷脂抗体阳性作为替代纳入标准，以及/或允许从多个器官使用较少的临床标准进行分类，提高了诊断时的敏感性（中位数为 82.0%和 86.2%）和整体敏感性（93.7%和 97.1%），特异性略有下降。重要的是，被修改后的标准遗漏的患者发生主要器官受累、使用免疫抑制剂/生物疗法和器官损害的发生率较低。
SLICC 和 EULAR/ACR 比 ACR 更敏感，EULAR/ACR 标准在早期 SLE 中的特异性更高，尽管可能会遗漏有显著疾病的患者。分类算法的组合和/或修改可以提高其敏感性，允许对更多高疾病负担的患者进行更早的分类和治疗。
