Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study
Gabriella E. Lube MD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorMariana Paes Leme Ferriani MD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorLucia Maria Arruda Campos MD, PhD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorMaria Teresa Terreri MD, PhD
Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorEloisa Bonfá MD, PhD
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Search for more papers by this authorClaudia Saad Magalhães MD, PhD
São Paulo State University (UNESP), Faculdade de Medicina de Botucatu, Botucatu, São Paulo - Brazil
Search for more papers by this authorNadia Emi Aikawa MD, PhD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Search for more papers by this authorDaniela Petry Piotto MD, PhD
Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorOctavio Augusto Bedin Peracchi MD, PhD
Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorMaria Carolina dos Santos MD, PhD
Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo city, São Paulo - Brazil
Search for more papers by this authorSimone Appenzeller MD, PhD
Rheumatology Unit, Faculty of Medical Sciences, State University of Campinas, Campinas, São Paulo - Brazil
Search for more papers by this authorVirginia Paes Leme Ferriani MD, PhD
Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo - Brazil
Search for more papers by this authorRosa Maria Rodrigues Pereira MD, PhD
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Search for more papers by this authorCorresponding Author
Clovis Artur Silva MD, PhD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Correspondence to: Prof. Clovis Artur Silva, Av. Dr. Eneas Carvalho Aguiar 647, Cerqueira César, São Paulo, São Paulo, Brazil, CEP 05403-000; Phone: 55-11-2661-8563; FAX 55-11-2661-8503, E-mail: [email protected]
Search for more papers by this authorGabriella E. Lube MD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorMariana Paes Leme Ferriani MD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorLucia Maria Arruda Campos MD, PhD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorMaria Teresa Terreri MD, PhD
Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorEloisa Bonfá MD, PhD
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Search for more papers by this authorClaudia Saad Magalhães MD, PhD
São Paulo State University (UNESP), Faculdade de Medicina de Botucatu, Botucatu, São Paulo - Brazil
Search for more papers by this authorNadia Emi Aikawa MD, PhD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Search for more papers by this authorDaniela Petry Piotto MD, PhD
Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorOctavio Augusto Bedin Peracchi MD, PhD
Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil
Search for more papers by this authorMaria Carolina dos Santos MD, PhD
Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo city, São Paulo - Brazil
Search for more papers by this authorSimone Appenzeller MD, PhD
Rheumatology Unit, Faculty of Medical Sciences, State University of Campinas, Campinas, São Paulo - Brazil
Search for more papers by this authorVirginia Paes Leme Ferriani MD, PhD
Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo - Brazil
Search for more papers by this authorRosa Maria Rodrigues Pereira MD, PhD
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Search for more papers by this authorCorresponding Author
Clovis Artur Silva MD, PhD
Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil
Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil
Correspondence to: Prof. Clovis Artur Silva, Av. Dr. Eneas Carvalho Aguiar 647, Cerqueira César, São Paulo, São Paulo, Brazil, CEP 05403-000; Phone: 55-11-2661-8563; FAX 55-11-2661-8503, E-mail: [email protected]
Search for more papers by this authorGrant sponsor: Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 303422/2015-7); Grant sponsor: Federico Foundation; Grant sponsor: Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” da USP (NAP-CriAd).
Conflict of interest: Nothing to declare.
Abstract
Background
Evans syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) patients has been rarely reported and limited to small populations.
Procedures
A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services including 850 patients with cSLE. ES was assessed at disease diagnosis and defined by the combination of immune thrombocytopenia and autoimmune hemolytic anemia.
Results
ES was observed in 11 of 850 (1.3%) cSLE patients. The majority of them had hemorrhagic manifestations (91%) and active disease (82%). All patients with ES were hospitalized and none died. Comparisons of cSLE patients with and without ES at diagnosis revealed similar frequencies of female gender, multiorgan involvement, autoantibodies profile, and low complement (P > 0.05). Patients with ES had a lower frequency of malar rash (9% vs. 53%, P = 0.003) and musculoskeletal involvement (18% vs. 69%, P = 0.001) than those without this complication. The frequencies of intravenous methylprednisolone (82% vs. 43%, P = 0.013) and intravenous immunoglobulin use (64% vs. 3%, P < 0.0001) were significantly higher in the ES group, with similar current prednisone dose between groups (1.1 [0.76–1.5] vs. 1.0 mg/kg/day [0–30], P = 0.195).
Conclusions
Our large multicenter study identified ES as a rare and severe initial manifestation of active cSLE with good outcome. Diagnosis is challenging due to the lack of typical signs and symptoms of lupus and the requirement to exclude infection and primary immunodeficiency.
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