Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study

Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study

Author Lube, Gabriella E. Google Scholar
Leme Ferriani, Mariana Paes Google Scholar
Arruda Campos, Lucia Maria Google Scholar
Terreri, Maria Teresa Autor UNIFESP Google Scholar
Bonfa, Eloisa Google Scholar
Magalhaes, Claudia Saad Google Scholar
Aikawa, Nadia Emi Google Scholar
Piotto, Daniela Petry Autor UNIFESP Google Scholar
Bedin Peracchi, Octavio Augusto Autor UNIFESP Google Scholar
dos Santos, Maria Carolina Google Scholar
Appenzeller, Simone Google Scholar
Leme Ferriani, Virginia Paes Google Scholar
Rodrigues Pereira, Rosa Maria Google Scholar
Silva, Clovis Artur Google Scholar
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. (C) 2016 Wiley Periodicals, Inc.
Keywords childhood-onset systemic lupus erythematosus
Evans syndrome
multicenter study
xmlui.dri2xhtml.METS-1.0.item-coverage Hoboken
Language English
Date 2016
Published in Pediatric Blood & Cancer. Hoboken, v. 63, n. 7, p. 1238-1243, 2016.
ISSN 1545-5009 (Sherpa/Romeo, impact factor)
Publisher Wiley
Extent 1238-1243
Origin http://dx.doi.org/10.1002/pbc.25976
Access rights Closed access
Type Article
Web of Science ID WOS:000380105400016
URI https://repositorio.unifesp.br/handle/11600/57661

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