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2019 年短期累积:欧洲抗风湿病联盟/美国风湿病学会域和系统性红斑狼疮国际合作临床中心/美国风湿病学会在发病时有无肾炎的狼疮患者中的损害。

Short-term Accrual 2019 European League Against Rheumatism/American College of Rheumatology Domains and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage in Lupus Patients With and Without Nephritis at Disease Onset.

机构信息

From the Rheumatology Division.

Pediatric Rheumatology Unit, Children's Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.

出版信息

J Clin Rheumatol. 2023 Jun 1;29(4):190-195. doi: 10.1097/RHU.0000000000001939. Epub 2023 Jan 21.

Abstract

OBJECTIVE

To determine in a historical inception cohort the impact of lupus nephritis at disease onset in short-term accrual 2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) domains. The possible association with treatment and damage was also investigated.

METHODS

One hundred thirty-three consecutive adult systemic lupus erythematosus patients according to the 2019 EULAR/ACR criteria were divided according to the presence (RENAL-lupus) or absence of renal involvement (NONRENAL-lupus) at disease onset. The 2019 EULAR/ACR score and Systemic Lupus International Collaborating Clinics/ACR (SDI) were longitudinally evaluated over 3 years.

RESULTS

RENAL-lupus (n = 49 [36.8%]) and NONRENAL-lupus (n = 84 [63.2%]) were similar regarding age ( p = 0.704), female sex ( p = 0.313), and black race ( p = 0.506). At study entry, RENAL-lupus had higher 2019 EULAR/ACR total domains (30 [12-42] vs. 22 [10-36], p < 0.001) and used more often glucocorticoid ( p < 0.001), mycophenolate mofetil ( p = 0.007), and cyclophosphamide ( p = 0.001). After 3 years, a stable number of domain scores was observed for the RENAL-lupus (30 [12-42] vs. 30 [12-42], p = 0.125), whereas an increase was observed for the NONRENAL-lupus (22 [10-36] vs. 23 [10-40], p < 0.001) compared with baseline. Accordingly, RENAL-lupus patients had a lower frequency of additional domains (3/49 [6.1%] vs. 37/84 [44.0%], p < 0.0001). New kidney involvement occurred in 15 (44.1%) of 34 patients of the NONRENAL-lupus. Both groups evolved with a comparable increase in frequency of patients with damage (SDI ≥1) at the end of the study (23/49 [46.9%] vs. 34/89 [40.54%], p = 0.585) with a similar median of SDI (1 [0-4] vs. 0 [0-2], p = 0.132).

CONCLUSIONS

The distinct pattern of accrual 2019 EULAR/ACR domains in patients with and without nephritis at disease onset suggests that close surveillance for additional organ involvement, including kidney, is mandatory in NONRENAL lupus in the first 3 years of disease. The unexpected comparable early damage in both groups despite milder disease and less intense immunosuppression in NONRENAL lupus reinforces the need for new and tailored therapies for these patients.

摘要

目的

在一项历史发病队列中,确定狼疮肾炎在短期 2019 年欧洲抗风湿病联盟(EULAR)/美国风湿病学会(ACR)域中的发病时对短期结果的影响。还研究了与治疗和损害的可能关联。

方法

根据 2019 年 EULAR/ACR 标准,将 133 例连续的成人系统性红斑狼疮患者分为有(RENL-狼疮)或无(NONRENAL-狼疮)肾脏受累时发病。对 2019 年 EULAR/ACR 评分和系统性红斑狼疮国际协作诊所/ACR(SDI)进行了 3 年的纵向评估。

结果

RENL-狼疮(n=49[36.8%])和 NONRENAL-狼疮(n=84[63.2%])在年龄(p=0.704)、女性(p=0.313)和黑种人(p=0.506)方面相似。在研究开始时,RENL-狼疮的 2019 年 EULAR/ACR 总域评分更高(30[12-42]比 22[10-36],p<0.001),并且更常使用糖皮质激素(p<0.001)、吗替麦考酚酯(p=0.007)和环磷酰胺(p=0.001)。3 年后,RENL-狼疮的域评分保持稳定(30[12-42]比 30[12-42],p=0.125),而 NONRENAL-狼疮的域评分增加(22[10-36]比 23[10-40],p<0.001)与基线相比。相应地,RENL-狼疮患者的附加域的频率较低(3/49[6.1%]比 37/84[44.0%],p<0.0001)。在 NONRENAL-狼疮中,有 15 名(44.1%)患者发生新的肾脏受累。两组患者在研究结束时均出现相似频率的患者发生损害(SDI≥1)(23/49[46.9%]比 34/89[40.54%],p=0.585),SDI 的中位数相似(1[0-4]比 0[0-2],p=0.132)。

结论

在发病时有和无肾炎的患者中 2019 年 EULAR/ACR 域的累积模式不同,这表明在疾病的前 3 年中,必须对 NONRENAL 狼疮患者进行密切监测,以发现其他器官的受累,包括肾脏。尽管 NONRENAL 狼疮患者的疾病较轻且免疫抑制作用较弱,但两组患者的早期损害均相当,这再次强调需要为这些患者制定新的、有针对性的治疗方法。

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