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dc.contributor.authorLube, Gabriella E.
dc.contributor.authorLeme Ferriani, Mariana Paes
dc.contributor.authorArruda Campos, Lucia Maria
dc.contributor.authorTerreri, Maria Teresa [UNIFESP]
dc.contributor.authorBonfa, Eloisa
dc.contributor.authorMagalhaes, Claudia Saad
dc.contributor.authorAikawa, Nadia Emi
dc.contributor.authorPiotto, Daniela Petry [UNIFESP]
dc.contributor.authorBedin Peracchi, Octavio Augusto [UNIFESP]
dc.contributor.authordos Santos, Maria Carolina
dc.contributor.authorAppenzeller, Simone
dc.contributor.authorLeme Ferriani, Virginia Paes
dc.contributor.authorRodrigues Pereira, Rosa Maria
dc.contributor.authorSilva, Clovis Artur
dc.identifier.citationPediatric Blood & Cancer. Hoboken, v. 63, n. 7, p. 1238-1243, 2016.
dc.description.abstractBackground. 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. (C) 2016 Wiley Periodicals, Inc.en
dc.relation.ispartofPediatric Blood & Cancer
dc.rightsAcesso restrito
dc.subjectchildhood-onset systemic lupus erythematosusen
dc.subjectEvans syndromeen
dc.subjectmulticenter studyen
dc.titleEvans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Studyen
dc.description.affiliationUniv Sao Paulo, FM, Pediat Rheumatol Unit, Sao Paulo, Brazil
dc.description.affiliationUniv Fed Sao Paulo UNIFESP, Pediat Rheumatol Unit, Sao Paulo, Brazil
dc.description.affiliationFMUSP, Div Rheumatol, Sao Paulo, Brazil
dc.description.affiliationSao Paulo State Univ UNESP, Fac Med Botucatu, Sao Paulo, Brazil
dc.description.affiliationIrmandade Santa Casa Misericordia Sao Paulo, Sao Paulo, Brazil
dc.description.affiliationUniv Estadual Campinas, Fac Med Sci, Rheumatol Unit, Sao Paulo, Brazil
dc.description.affiliationUniv Sao Paulo, Ribeirao Preto Med Sch, Sao Paulo, Brazil
dc.description.affiliationUnifespUniv Fed Sao Paulo UNIFESP, Pediat Rheumatol Unit, Sao Paulo, Brazil
dc.description.sourceWeb of Science

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