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

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 thi...

Full description

Saved in:

Bibliographic Details
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
Online Access:	Get full text
Tags:	Add Tag No Tags, Be the first to tag this record!

Description
Summary:	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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0961-2033 1477-0962
DOI:	10.1177/09612033231170515