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

• Published in: Pediatric blood & cancer Vol. 63; no. 7; pp. 1238 - 1243
• Main Authors: Lube, Gabriella E., Ferriani, Mariana Paes Leme, Campos, Lucia Maria Arruda, Terreri, Maria Teresa, Bonfá, Eloisa, Magalhães, Claudia Saad, Aikawa, Nadia Emi, Piotto, Daniela Petry, Peracchi, Octavio Augusto Bedin, dos Santos, Maria Carolina, Appenzeller, Simone, Ferriani, Virginia Paes Leme, Pereira, Rosa Maria Rodrigues, Silva, Clovis Artur
• Format: Journal Article
• Language: English
• Published: United States Blackwell Publishing Ltd 01-07-2016; Wiley Subscription Services, Inc
• Subjects: Adolescent; Adult; Anemia, Hemolytic, Autoimmune - blood; Anemia, Hemolytic, Autoimmune - diagnosis; Anemia, Hemolytic, Autoimmune - drug therapy; Anemia, Hemolytic, Autoimmune - etiology; Autoantibodies - blood; Child; Child, Preschool; childhood-onset systemic lupus erythematosus; Complement System Proteins - metabolism; Evans syndrome; Female; Hematology; Humans; Lupus; Lupus Erythematosus, Systemic - blood; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - drug therapy; Male; Methylprednisolone - administration & dosage; multicenter study; Oncology; Pediatrics; Retrospective Studies; Thrombocytopenia - blood; Thrombocytopenia - diagnosis; Thrombocytopenia - drug therapy; Thrombocytopenia - etiology; Evans syndrome; childhood-onset systemic lupus erythematosus; multicenter study
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.25976

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.