Sakamoto Ana P, Silva Clovis A, Pita Ana C, Trindade Vitor C, Islabao Aline G, Fiorot Fernanda J, Lopes Sandra Rm, Pereira Rosa Mr, Saad-Magalhaes Claudia, Russo Gleice Cs, Len Claudio A, Prado Rogerio do, Campos Lucia Ma, Aikawa Nadia E, Appenzeller Simone, Ferriani Virginia Pl, Silva Marco F, Felix Marta, Fonseca Adriana R, Assad Ana Pl, Sztajnbok Flavio R, Santos Maria C, Bica Blanca E, Sena Evaldo G, Moraes Ana J, Fraga Melissa M, Robazzi Teresa C, Spelling Paulo F, Scheibel Iloite M, Cavalcanti Andre S, Matos Erica N, Guimaraes Luciano J, Santos Flavia P, Mota Licia Mh, Bonfa Eloisa, Terreri Maria T
Pediatric Rheumatology Unit, Universidade Federal de Sao Paulo, Sao Paulo, SP, Brazil.
Pediatric Rheumatology Unit, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.
Lupus. 2023 May;32(6):781-790. doi: 10.1177/09612033231170515. Epub 2023 Apr 18.
To identify associations between mortality in cSLE patients and their characteristics: clinical and laboratory features, disease activity and damage scores, and treatment; to evaluate risk factors associated with mortality in cSLE; and to determine the most frequent causes of death in this group of patients.
We performed a multicenter retrospective cohort using data from 1,528 cSLE patients followed in 27 pediatric rheumatology tertiary centers in Brazil. Patients' medical records were reviewed according to a standardized protocol, in which information regarding demographic and clinical features, disease activity and damage scores, and treatment were collected and compared between deceased cSLE patients and survivors. Univariate and multivariate analyses by Cox regression model were used to calculate risk factors for mortality, whereas survival rates were analyzed by Kaplan-Meier plots.
A total of 63/1,528 (4.1%) patients deceased, 53/63 were female (84.1%), median age at death was 11.9 (9.4-13.1) years and median time interval between cSLE diagnosis and death was 3.2 (0.5-5.3) years. Sepsis was the main cause of death in 27/63 (42.8%) patients, followed by opportunistic infections in 7/63 (11.1%), and alveolar hemorrhage in 6/63 (9.5%) patients. The regression models resulted in neuropsychiatric lupus (NP-SLE) (HR = 2.56, 95% CI = 1.48-4.42) and chronic kidney disease (CKD) (HR = 4.33, 95% CI = 2.33-4.72), as risk factors significantly associated with mortality. Overall patient survival after cSLE diagnosis at 5, 10, and 15 years were 97%, 95.4%, and 93.8%, respectively.
This study confirmed that the recent mortality rate in cSLE in Brazil is low, but still of concern. NP-SLE and CKD were the main risk factors for mortality, indicating that the magnitude of these manifestations was significantly high.
确定儿童系统性红斑狼疮（cSLE）患者的死亡率与其特征之间的关联，这些特征包括临床和实验室检查结果、疾病活动度和损伤评分以及治疗情况；评估与cSLE患者死亡率相关的危险因素；并确定该组患者最常见的死亡原因。
我们进行了一项多中心回顾性队列研究，使用了巴西27个儿科风湿病三级中心随访的1528例cSLE患者的数据。根据标准化方案对患者的病历进行审查，收集有关人口统计学和临床特征、疾病活动度和损伤评分以及治疗的信息，并在已故cSLE患者和幸存者之间进行比较。采用Cox回归模型进行单因素和多因素分析以计算死亡率的危险因素，而生存率则通过Kaplan-Meier曲线进行分析。
共有63/1528例（4.1%）患者死亡，53/63例为女性（84.1%），死亡时的中位年龄为11.9岁（9.4 - 13.1岁），cSLE诊断至死亡的中位时间间隔为3.2年（0.5 - 5.3年）。脓毒症是27/63例（42.8%）患者的主要死亡原因，其次是机会性感染7/63例（11.1%），肺泡出血6/63例（9.5%）。回归模型显示神经精神性狼疮（NP-SLE）（风险比[HR]=2.56，95%置信区间[CI]=1.48 - 4.42）和慢性肾脏病（CKD）（HR = 4.33，95% CI = 2.33 - 4.72）是与死亡率显著相关的危险因素。cSLE诊断后5年、10年和15年的总体患者生存率分别为97%、95.4%和93.8%。
本研究证实巴西近期cSLE患者的死亡率较低，但仍令人担忧。NP-SLE和CKD是主要的死亡危险因素，表明这些表现的严重程度显著较高。
