Evans syndrome at childhood‐onset systemic lupus erythematosus diagnosis : a large multicenter study
Gabriella E. Lube, Mariana Paes Leme Ferriani, Lucia Maria Arruda Campos, Maria Teresa Terreri, Eloisa Bonfá, Claudia Saad Magalhães, Nadia Emi Aikawa, Daniela Petry Piotto, Octavio Augusto Bedin Peracchi, Maria Carolina dos Santos, Simone Appenzeller, Virginia Paes Leme Ferriani, Rosa Maria...
Gabriella E. Lube, Mariana Paes Leme Ferriani, Lucia Maria Arruda Campos, Maria Teresa Terreri, Eloisa Bonfá, Claudia Saad Magalhães, Nadia Emi Aikawa, Daniela Petry Piotto, Octavio Augusto Bedin Peracchi, Maria Carolina dos Santos, Simone Appenzeller, Virginia Paes Leme Ferriani, Rosa Maria Rodrigues Pereira, Clovis Artur Silva
ARTIGO
Inglês
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...
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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.
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CONSELHO NACIONAL DE DESENVOLVIMENTO CIENTÍFICO E TECNOLÓGICO - CNPQ
303422/2015‐7
Fechado
Evans syndrome at childhood‐onset systemic lupus erythematosus diagnosis : a large multicenter study
Gabriella E. Lube, Mariana Paes Leme Ferriani, Lucia Maria Arruda Campos, Maria Teresa Terreri, Eloisa Bonfá, Claudia Saad Magalhães, Nadia Emi Aikawa, Daniela Petry Piotto, Octavio Augusto Bedin Peracchi, Maria Carolina dos Santos, Simone Appenzeller, Virginia Paes Leme Ferriani, Rosa Maria...
Gabriella E. Lube, Mariana Paes Leme Ferriani, Lucia Maria Arruda Campos, Maria Teresa Terreri, Eloisa Bonfá, Claudia Saad Magalhães, Nadia Emi Aikawa, Daniela Petry Piotto, Octavio Augusto Bedin Peracchi, Maria Carolina dos Santos, Simone Appenzeller, Virginia Paes Leme Ferriani, Rosa Maria Rodrigues Pereira, Clovis Artur Silva
Evans syndrome at childhood‐onset systemic lupus erythematosus diagnosis : a large multicenter study
Gabriella E. Lube, Mariana Paes Leme Ferriani, Lucia Maria Arruda Campos, Maria Teresa Terreri, Eloisa Bonfá, Claudia Saad Magalhães, Nadia Emi Aikawa, Daniela Petry Piotto, Octavio Augusto Bedin Peracchi, Maria Carolina dos Santos, Simone Appenzeller, Virginia Paes Leme Ferriani, Rosa Maria...
Gabriella E. Lube, Mariana Paes Leme Ferriani, Lucia Maria Arruda Campos, Maria Teresa Terreri, Eloisa Bonfá, Claudia Saad Magalhães, Nadia Emi Aikawa, Daniela Petry Piotto, Octavio Augusto Bedin Peracchi, Maria Carolina dos Santos, Simone Appenzeller, Virginia Paes Leme Ferriani, Rosa Maria Rodrigues Pereira, Clovis Artur Silva
Fontes
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Pediatric blood & cancer Vol. 63, no. 7 (July, 2016), p. 1238-1243 |