Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.
Division of Rheumatology, 117265Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.
Lupus. 2021 Dec;30(14):2286-2291. doi: 10.1177/09612033211054397. Epub 2021 Oct 25.
To evaluate if the 2019-European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria at diagnosis of childhood-onset systemic lupus erythematosus (cSLE) are associated with higher rates of early damage scored by Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI).
This retrospective multicenter study included 670 cSLE patients with ≤5 years of disease duration. All patients fulfilled both 2019-EULAR/ACR and 1997-ACR classification criteria. Total score of 2019-EULAR/ACR criteria and each of its specific domains were assessed at diagnosis as predictors of damage accrual at the last visit, according to the presence of any organ damage (defined by SDI ≥ 1).
Median disease duration was 2.8 (IQR 1.8-3.8) years and 200 (29.9%) patients had at least one organ damage (SDI ≥ 1). The most frequent domains were neuropsychiatric (12%), renal (7%), and musculoskeletal (6%). There was a higher frequency of renal (58% vs 43%, = 0.0004) and neuropsychiatric domain (21% vs 7%, < 0.0001) of 2019-EULAR/ACR criteria in patients with damage (SDI ≥ 1) compared to those without damage (SDI = 0). Patients scoring renal or neuropsychiatric domains of the 2019-EULAR/ACR criteria at diagnosis were associated with renal damage (odds ratio 9.701, 95% confidence interval 3.773-24.941, < 0.001) or neuropsychiatric damage (OR 9.480, 95% CI 5.481-16.399, <0.0001) at latest visit, respectively. cSLE patients with positive anti-dsDNA at diagnosis were also associated with renal damage by the latest visit (OR 2.438, 95% CI 1.114-5.3381, = 0.021). Constitutional, hematologic, mucocutaneous, serosal, and musculoskeletal domains and specific criteria as well as other immunologic criteria were not associated with damage accrual. Median of SLEDAI-2K was significantly higher in patients with global damage (19.5 (2-51) vs 14 (0-51), <0.001). 2019-EULAR/ACR score >25 was associated with more overall (SDI ≥ 1) (38% vs 25%, = 0.0002) and renal damage (11% vs 5%, = 0.023).
The 2019-EULAR/ACR criteria at diagnosis were associated with a higher rate of early damage in cSLE patients, especially for renal and neuropsychiatric damage. Of note, damage was particularly associated with high disease activity at diagnosis and 2019-EULAR/ACR score >25.
评估 2019 年欧洲抗风湿病联盟/美国风湿病学会（EULAR/ACR）儿童发病系统性红斑狼疮（cSLE）诊断标准是否与更高的累积系统红斑狼疮国际合作临床/美国风湿病学会（SLICC/ACR）损伤指数（SDI）评分的早期损伤率相关。
本回顾性多中心研究纳入了 670 名病程≤5 年的 cSLE 患者。所有患者均符合 2019 年 EULAR/ACR 和 1997 年 ACR 分类标准。在最后一次就诊时，根据是否存在任何器官损伤（SDI≥1），评估 2019 年 EULAR/ACR 标准的总评分和其每个特定领域作为损伤累积的预测因素。
中位病程为 2.8（IQR 1.8-3.8）年，200（29.9%）名患者至少存在一项器官损伤（SDI≥1）。最常见的领域是神经精神（12%）、肾脏（7%）和肌肉骨骼（6%）。与无损伤（SDI=0）患者相比，有损伤（SDI≥1）的患者 2019 年 EULAR/ACR 标准的肾脏（58% vs 43%，=0.0004）和神经精神域（21% vs 7%，<0.0001）的发生率更高。在诊断时患有 2019 年 EULAR/ACR 标准的肾脏或神经精神疾病的患者与肾脏损伤（优势比 9.701，95%置信区间 3.773-24.941，<0.001）或神经精神损伤（OR 9.480，95%CI 5.481-16.399，<0.0001）相关，分别为最新就诊时的损伤。在诊断时抗 dsDNA 阳性的 cSLE 患者也与最新就诊时的肾脏损伤相关（OR 2.438，95%CI 1.114-5.3381，=0.021）。固有、血液、黏膜皮肤、浆膜和肌肉骨骼域和特定标准以及其他免疫标准与损伤累积无关。全球损伤患者的 SLEDAI-2K 中位数明显更高（19.5（2-51）比 14（0-51），<0.001）。2019 年 EULAR/ACR 评分>25 与更多的总体（SDI≥1）（38% vs 25%，=0.0002）和肾脏损伤（11% vs 5%，=0.023）相关。
在 cSLE 患者中，诊断时的 2019 年 EULAR/ACR 标准与更高的早期损伤率相关，尤其是与肾脏和神经精神损伤相关。值得注意的是，损伤与诊断时的高疾病活动度和 2019 年 EULAR/ACR 评分>25 尤其相关。
