Agradecimentos: Our gratitude to Mitty Koyama and Ulysses Doria-Filho for the statistical analyses. The authors thank all the participants of the Pediatric Rheumatology Divisions and colleagues for including their patients: Pediatric Rheumatology Unit, UNIFESP (Daniela P Piotto), Pediatric Rheumatology Unit, ICr - FMUSP (Glaucia Novak, Beatriz Molinari, Adriana EM Salum, Katia T Kozu); Pediatric Rheumatology Unit, Division of Rheumatology, FMUSP; Pediatric Rheumatology Division, Sao Paulo State University, UNESP (Taciana AP Fernandes); Irmandade da Santa Casa de Sao Paulo (Silvana B Sacchetti); Pediatric Rheumatology Unit, State University of Campinas, UNICAMP (Roberto Marini); Ribeirao Preto Medical School - University of Sao Paulo (Luciana M Carvalho, Hugo Gomes, Paola P Kahwage); Hospital Municipal Infantil Menino Jesus (Simone Lotufo, Tania CM Castro); Pontifical Catholic University of Sorocaba (Valeria C Ramos); Instituto de Puericultura e Pediatria Matargao Gesteira/Universidade Federal do Rio de Janeiro (Sheila K Oliveira); Hospital Municipal Piedade, Rio de Janeiro (Cynthia T Silva); Hospital Geral de Fortaleza, Fortaleza (Carlos N Rabelo-Junior, Ana Raquel Feitosa); Hospital Albert Sabin (Luciana B Paim); Federal University of Pernambuco; Obras Sociais Irma Dulce, Salvador (Ana M Rolim); Hospital Jose Alencar, Brasilia (Maria CM Ribeiro). The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was supported by the Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (2015/03756-4), Coordenacao de Aperfeicoamento de Pessoal de Nivel Superior, Nucleo de Apoio a Pesquisa "Saude da Crianca e do Adolescente" da USP (NAP-CriAd), Conselho Nacional de Desenvolvimento Cientifico e Tecnologico (301479/2015-1, 303752/2015-7, 304984/2020-5, 305242/2019-9, 305556/2017-7) Ver menos

Abstract: 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 Ver menos