避免将血栓性原发性抗磷脂综合征误诊为系统性红斑狼疮（SLE）：哪些是表现最佳的 SLE 分类标准？
Avoiding misclassification of thrombotic primary antiphospholipid syndrome as systemic lupus erythematosus (SLE): What are the best-performing SLE classification criteria?
机构信息
Rheumatology Division, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
Rheumatology Division, Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
出版信息
Lupus. 2021 Oct;30(11):1732-1738. doi: 10.1177/09612033211033978. Epub 2021 Jul 22.
BACKGROUND
Systemic lupus erythematosus (SLE) and Primary Antiphospholipid Syndrome (PAPS) overlap clinical and immunological features. Therefore, misclassification of PAPS patients as SLE is a concern. The ACR/EULAR 2019 SLE classification has never been studied in PAPS.
OBJECTIVE
To verify if the ACR/EULAR 2019 SLE classification can correctly classify a PAPS patient as not having SLE and compare its performance with the SLICC 2012 SLE classification. Methods: One-hundred thrombotic PAPS patients who fulfilled the Sidney criteria were consecutively screened and those who attended the inclusion criteria were submitted to ACR/EULAR 2019 and SLICC 2012 classifications.
RESULTS
Sixty-seven PAPS patients were included in this study. The majority was female (89.6%) with median age at study inclusion of 45 years (35-53) and median PAPS disease duration of 13 years (8-19). PAPS correct classification was observed more often with ACR/EULAR 2019 than SLICC 2021 criteria (94.0% vs. 64.2%; p < 0.001). The 4 misclassified patients in ACR/EULAR 2019 were also misclassified in SLICC 2012. The comparison of misclassified patients to those correctly not classified as SLE resulted, for both criteria, in higher frequencies of hematological domain [ACR/EULAR 2019 (100% vs. 28.6%, p = 0.010) and SLICC 2012 (95.8% vs. 11.6%, p < 0.001)]. Further analysis of hematological manifestations revealed that for the ACR/EULAR 2019 leukopenia (100% vs. 22.2%, p = 0.004) and for the SLICC 2012 leukopenia/lymphopenia (91.7% vs. 7%, p < 0.001) were more frequent in misclassified group. Proteinuria (20.8% vs. 0%, p = 0.004) and low complement (45.8% vs. 20.9%, p = 0.033) were also more often observed in the incorrectly SLICC 2012 classified patients.
CONCLUSION
ACR/EULAR 2019 had high accuracy for distinguishing PAPS from SLE, whereas the SLICC 2012 incorrectly classified more than one third of the PAPS patients as having SLE.
背景
系统性红斑狼疮（SLE）和原发性抗磷脂综合征（PAPS）在临床表现和免疫学特征上存在重叠。因此，将 PAPS 患者误诊为 SLE 是一个需要关注的问题。ACR/EULAR 2019 年 SLE 分类标准从未在 PAPS 患者中进行过研究。
目的
验证 ACR/EULAR 2019 年 SLE 分类标准是否能正确地将 PAPS 患者归类为非 SLE，并比较其与 SLICC 2012 SLE 分类标准的性能。
方法
连续筛选了 100 例符合 Sidney 标准的血栓性 PAPS 患者，符合纳入标准的患者接受了 ACR/EULAR 2019 年和 SLICC 2012 年分类标准的评估。
结果
本研究共纳入 67 例 PAPS 患者，其中大多数为女性（89.6%），中位年龄为 45 岁（35-53 岁），中位 PAPS 病程为 13 年（8-19 年）。与 SLICC 2012 标准相比，ACR/EULAR 2019 标准更能正确地将 PAPS 患者归类为非 SLE（94.0% vs. 64.2%；p<0.001）。在 ACR/EULAR 2019 标准中被错误归类的 4 例患者在 SLICC 2012 标准中也被错误归类。与正确归类为非 SLE 的患者相比，两种标准的错误归类患者的血液学表现更为常见（ACR/EULAR 2019 标准为 100% vs. 28.6%，p=0.010；SLICC 2012 标准为 95.8% vs. 11.6%，p<0.001）。进一步分析血液学表现发现，ACR/EULAR 2019 标准中白细胞减少症（100% vs. 22.2%，p=0.004）和 SLICC 2012 标准中白细胞减少症/淋巴细胞减少症（91.7% vs. 7%，p<0.001）在错误归类组中更为常见。在不正确地被 SLICC 2012 标准归类为 SLE 的患者中，蛋白尿（20.8% vs. 0%，p=0.004）和低补体（45.8% vs. 20.9%，p=0.033）也更为常见。
结论
ACR/EULAR 2019 标准对区分 PAPS 和 SLE 具有较高的准确性，而 SLICC 2012 标准则错误地将三分之一以上的 PAPS 患者归类为 SLE。
Zotero 插件