Risk factors for mortality in 1528 Brazilian childhood-onset systemic lupus erythematosus patients

• Published in: Lupus Vol. 32; no. 6; pp. 781 - 790
• Main Authors: 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
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01-05-2023; Sage Publications Ltd
• Subjects: Age of Onset; Alveoli; Brazil - epidemiology; Child; Children; Death; Diagnosis; Female; Hemorrhage; Humans; Kidney diseases; Lupus; Lupus Erythematosus, Systemic - complications; Male; Medical records; Mortality; Neurological complications; Patients; Pediatrics; Regression analysis; Renal Insufficiency, Chronic - complications; Retrospective Studies; Rheumatology; Risk Factors; Sepsis; Survival; Systemic lupus erythematosus; Brazil; mortality; lupus nephritis; childhood-onset systemic lupus erythematosus; sepsis; Childhood
• ISSN: 0961-2033; 1477-0962
• DOI: 10.1177/09612033231170515

Objectives
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.
Methods
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.
Results
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.
Conclusions
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.