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

Lube, Gabriella E.; Ferriani, Mariana Paes Leme; Campos, Lúcia Maria Arruda; Terreri, Maria Teresa; Bonfá, Eloísa; Magalhães, Cláudia Saad; Aikawa, Nádia E.; Piotto, Daniela Gerent Petry; Peracchi, Octávio Augusto Bedin; Santos, Maria Carolina dos; Appenzeller, Simone; Ferriani, Virgínia Paes Leme; Pereira, Rosa Maria Rodrigues; Silva, Clóvis A.

doi:10.1002/pbc.25976

Cited by 21 publications

(27 citation statements)

References 47 publications

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“…The incidence of ES in children is unknown due to a lack of published studies, with the few available consisting of case reports or retrospective cohorts. 6 , 12 , 59 , 74 – 76 From the largest cohorts of autoimmune cytopenias, it can be concluded that ES is more frequent during childhood; in a study, 11 (4.1%) of 179 children had ES 6 vs 6 (0.78%) of 766 adults. 77 It is important to note that autoimmune cytopenias in children are frequently associated with primary immunodeficiencies and in this group, they should always be ruled out.…”

Section: Classificationmentioning

confidence: 99%

“…Several studies have demonstrated the relationship between SLE and ES; 9 , 10 , 58 prevalence of secondary ES in SLE has been reported in 1.7%–2.7% of cases. 24 , 59 Hemorrhagic manifestations are frequently observed in this group, including petechiae, ecchymosis, epistaxis, gingivorraghia, gastrointestinal bleeding and hematuria. 5 , 59 Spontaneous splenic rupture as an initial manifestation of ES has been documented, 60 confirming the heterogeneous course of the disease.…”

Section: Classificationmentioning

confidence: 99%

“… 24 , 59 Hemorrhagic manifestations are frequently observed in this group, including petechiae, ecchymosis, epistaxis, gingivorraghia, gastrointestinal bleeding and hematuria. 5 , 59 Spontaneous splenic rupture as an initial manifestation of ES has been documented, 60 confirming the heterogeneous course of the disease. Steroids are the suggested first line of therapy.…”

Section: Classificationmentioning

confidence: 99%

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Evans syndrome: clinical perspectives, biological insights and treatment modalities

Jaime‐Pérez¹,

Aguilar-Calderón²,

Salazar‐Cavazos³

et al. 2018

JBM

113

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Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%–73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person’s lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Recent molecular theories explaining the physiopathology of ES include deficiencies of CTLA-4, LRBA, TPP2 and a decreased CD4/CD8 ratio. As in other autoimmune cytopenias, there is no established evidence-based treatment and steroids are the first-line therapy, with intravenous immunoglobulin administered as a life-saving resource in cases of severe immune thrombocytopenic purpura manifestations. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful, although it is necessary to consider its potential serious adverse effects. In conclusion, ES is a disease with a heterogeneous course that remains challenging to patients and physicians, with prospective clinical trials needed to explore potential targeted therapy to achieve an improved long-term response or even a cure.

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Section: Classificationmentioning

confidence: 99%

Section: Classificationmentioning

confidence: 99%

Section: Classificationmentioning

confidence: 99%

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Evans syndrome: clinical perspectives, biological insights and treatment modalities

Jaime‐Pérez¹,

Aguilar-Calderón²,

Salazar‐Cavazos³

et al. 2018

JBM

113

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“…LA includes 33 countries and dependencies, a large population, two main languages (Spanish and Portuguese), with distinct socio-economic and political status. Two groups have reported data focused on epidemiology, clinical and laboratorial features of cSLE patients in LA: BRAC-SLE (Brazilian Childhood-onset SLE Registry Group) [2][3][4][5][6][7][8][9][10][11][12][13][14][15] and GLADEL (Grupo Latino Americano De Estudio del Lupus) [16][17][18][19].…”

Section: Introductionmentioning

confidence: 99%

Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America

et al. 2018

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To assess epidemiology and management practices of Latin America Pediatric Rheumatologists (LAPR) about childhood-onset systemic lupus erythematosus (cSLE). A cross-sectional study was performed in 288 LAPR PANLAR members based on online survey about cSLE practices. The response rate of web-based survey by LAPR was 170/288(59%) and the majority worked in university hospitals (63%). The ACR and/or SLICC classification criteria (99%) and disease activity tools (97%) were almost universally used by LAPR, whereas damage index (70%) and CHAQ (58%) instruments were less frequently used. Laboratory exams, diagnostic imaging, and biopsies were generally available (> 75%), however low availability for densitometry (66%). Drug access was excellent for the most common prescribed medications (> 75%), except for belimumab (11%). Emerging mosquito-borne diseases were also reported: dengue (20%), chikungunya (11%), and Zika (8%). Groups were further divided in two, according to the median number of cSLE patients followed by LAPR in the last year: groups A and B (≥ 25 and < 25, respectively). Frequencies of condom in combination with other contraceptive methods were significantly higher in group A than B (p = 0.01). The frequencies of reported pregnancy (p < 0.001) and non-adherence to therapy were significantly higher in group A (p = 0.023). Alcohol intake (p = 0.004) and illicit drug use (p = 0.007) were also reported more frequently by LAPR of group A in at least one cSLE patient. This first large web-based survey demonstrated an overall excellent access for diagnosis and therapy by LAPR, probably related to their high rate of practices in tertiary care of university hospitals. Adherence to therapy, pregnancy, and substance abuse was identified as major challenges in this population, particularly in larger centers.

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“…2,16,17 The longest time interval to diagnosis observed in the present study occurred in a patient with hematological complications, also reported in other studies. 18…”

Section: Discussionmentioning

confidence: 99%

Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study

Novak

Molinari

Ferreira

et al. 2018

Lupus

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Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.

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

Abstract: 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.

Cited by 21 publications

References 47 publications

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Epidemiology and management practices for childhood-onset systemic lupus erythematosus patients: a survey in Latin America

Characteristics of 1555 childhood-onset lupus in three groups based on distinct time intervals to disease diagnosis: a Brazilian multicenter study

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