title: Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study.
abstract: Evans syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) patients has been rarely reported and limited to small populations.
  
  
  
  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.
  
  
  
  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).
  
  
  
  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.
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil.
authors.forename: Gabriella E
authors.initials: GE
authors.lastname: Lube
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil.
authors.forename: Mariana Paes Leme
authors.initials: MP
authors.lastname: Ferriani
authors.affiliation: Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo city, São Paulo - Brazil.
authors.forename: Lucia Maria Arruda
authors.initials: LM
authors.lastname: Campos
authors.affiliation: Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil.
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Terreri
authors.affiliation: Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Bonfá
authors.affiliation: São Paulo State University (UNESP), Faculdade de Medicina de Botucatu, Botucatu, São Paulo - Brazil.
authors.forename: Claudia Saad
authors.initials: CS
authors.lastname: Magalhães
authors.affiliation: 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.
authors.forename: Nadia Emi
authors.initials: NE
authors.lastname: Aikawa
authors.affiliation: Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil.
authors.forename: Daniela Petry
authors.initials: DP
authors.lastname: Piotto
authors.affiliation: Pediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo city, São Paulo - Brazil.
authors.forename: Octavio Augusto Bedin
authors.initials: OA
authors.lastname: Peracchi
authors.affiliation: Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo city, São Paulo - Brazil.
authors.forename: Maria Carolina
authors.initials: MC
authors.lastname: Dos Santos
authors.affiliation: Rheumatology Unit, Faculty of Medical Sciences, State University of Campinas, Campinas, São Paulo - Brazil.
authors.forename: Simone
authors.initials: S
authors.lastname: Appenzeller
authors.affiliation: Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo - Brazil.
authors.forename: Virginia Paes Leme
authors.initials: VP
authors.lastname: Ferriani
authors.affiliation: Division of Rheumatology, FMUSP, São Paulo city, São Paulo - Brazil.
authors.forename: Rosa Maria Rodrigues
authors.initials: RM
authors.lastname: Pereira
authors.affiliation: 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.
authors.forename: Clovis Artur
authors.initials: CA
authors.lastname: Silva
mesh_terms: D000293:Adolescent; D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D001323:Autoantibodies / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003165:Complement System Proteins / Q000378:metabolism; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications / Q000188:drug therapy; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage*; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology
chemical_list: D001323:Autoantibodies; D003165:Complement System Proteins; D008775:Methylprednisolone
keywords: Evans syndrome; childhood-onset systemic lupus erythematosus; multicenter study