Brief Report: How Do Patients With Newly Diagnosed Systemic Lupus Erythematosus Present? A Multicenter Cohort of Early Systemic Lupus Erythematosus to Inform the Development of New Classification Criteria

Brief Report: How Do Patients With Newly Diagnosed Systemic Lupus Erythematosus Present? A Multicenter Cohort of Early Systemic Lupus Erythematosus to Inform the Development of New Classification Criteria
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DOI:
10.1002/art.40674
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发表时间:
2019-01-01
影响因子:
13.3
通讯作者:
Touma, Zahi
Touma, Zahi
中科院分区:
医学1区
文献类型:
--
作者:
Mosca, Marta;Costenbader, Karen H.;Touma, Zahi

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目的 系统性红斑狼疮 (SLE) 会出现其他病症中也常见的非特异性体征和症状。本研究旨在评估疾病发作时的表现,并将早期 SLE 表现与类似 SLE 的疾病的表现进行比较。方法 亚洲、欧洲、北美和南美的学术狼疮中心收集了过去 3 年内因可能患有 SLE 而转诊至该中心的患者、有 SLE 症状持续时间的患者以及被诊断为患有类似 SLE 病症的患者的基线数据。对 1997 年美国风湿病学会 (ACR) SLE 分类标准和 2012 年系统性狼疮国际合作诊所 (SLICC) SLE 分类标准的诊断性能进行了测试。结果 收集了 389 名早期 SLE 患者和 227 名类似 SLE 病症患者的数据。不明原因发热在早期 SLE 中比在模拟 SLE 情况下更常见(分别为 34.5% 和 13.7%;P < 0.001)。早期 SLE 中不太常见的特征包括雷诺现象(22.1% vs 48.5%;P < 0.001)、干燥症状(4.4% vs 34.4%;P < 0.001)、吞咽困难(0.3% vs 6.2%;P < 0.001)和疲劳(28.3% vs 37.0%;P = 0.024)。抗双链 DNA、抗 β(2)-糖蛋白 I 抗体、Coombs 试验结果阳性、自身免疫性溶血性贫血、低补体血症和白细胞减少症在早期 SLE 中比在模拟 SLE 的情况下更常见。 ACR 和 SLICC 分类标准中详述的症状在早期 SLE 患者中明显更为常见。与 ACR 标准相比,使用 SLICC 标准未被识别为早期 SLE 的早期 SLE 患者较少(16.5% 对 33.9%),但 ACR 标准比 SLICC 标准表现出更高的特异性(91.6% 对 82.4%)。结论 在这个多中心队列中,确定了有助于区分早期 SLE 和模仿 SLE 病症的临床表现。这些发现可能有助于早期 SLE 诊断,并为正在进行的修订 SLE 分类标准的举措提供信息。
Objective Systemic lupus erythematosus (SLE) presents with nonspecific signs and symptoms that are also found in other conditions. This study aimed to evaluate manifestations at disease onset and to compare early SLE manifestations to those of diseases mimicking SLE. Methods Academic lupus centers in Asia, Europe, North America, and South America collected baseline data on patients who were referred to them during the previous 3 years for possible SLE and who had a symptom duration of SLE and those diagnosed as having SLE-mimicking conditions. Diagnostic performance of the 1997 American College of Rheumatology (ACR) SLE classification criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria was tested. Results Data were collected on 389 patients with early SLE and 227 patients with SLE-mimicking conditions. Unexplained fever was more common in early SLE than in SLE-mimicking conditions (34.5% versus 13.7%, respectively; P < 0.001). Features less common in early SLE included Raynaud's phenomenon (22.1% versus 48.5%; P < 0.001), sicca symptoms (4.4% versus 34.4%; P < 0.001), dysphagia (0.3% versus 6.2%; P < 0.001), and fatigue (28.3% versus 37.0%; P = 0.024). Anti-double-stranded DNA, anti-beta(2)-glycoprotein I antibodies, positive Coombs' test results, autoimmune hemolytic anemia, hypocomplementemia, and leukopenia were more common in early SLE than in SLE-mimicking conditions. Symptoms detailed in the ACR and SLICC classification criteria were significantly more frequent among those with early SLE. Fewer patients with early SLE were not identified as having early SLE with use of the SLICC criteria compared to the ACR criteria (16.5% versus 33.9%), but the ACR criteria demonstrated higher specificity than the SLICC criteria (91.6% versus 82.4%). Conclusion In this multicenter cohort, clinical manifestations that could help to distinguish early SLE from SLE-mimicking conditions were identified. These findings may aid in earlier SLE diagnosis and provide information for ongoing initiatives to revise SLE classification criteria.