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Type: Artigo
Title: Evans Syndrome At Childhood-onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study
Author: Lube
Gabriella E.; Leme Ferriani
Mariana Paes; Arruda Campos
Lucia Maria; Terreri
Maria Teresa; Bonfa
Eloisa; Magalhaes
Claudia Saad; Aikawa
Nadia Emi; Piotto
Daniela Petry; Bedin Peracchi
Octavio Augusto; dos Santos
Maria Carolina; Appenzeller
Simone; Leme Ferriani
Virginia Paes; Rodrigues Pereira
Rosa Maria; Silva
Clovis Artur
Abstract: 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.
Subject: Childhood-onset Systemic Lupus Erythematosus
Evans Syndrome
Multicenter Study
Editor: Wiley-Blackwell
Citation: Pediatric Blood & Cancer. Wiley-blackwell, v. 63, p. 1238 - 1243, 2016.
Rights: fechado
Identifier DOI: 10.1002/pbc.25976
Date Issue: 2016
Appears in Collections:Unicamp - Artigos e Outros Documentos

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