Childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome: A multicenter study with 1519 patients

Islabão, Aline Garcia; Mota, Lícia Maria Henrique da; Ribeiro, Maria Custódia Machado; Arabi, Tamima Mohamad Abou; Cividatti, Georgiana N; Queiroz, Lígia Bruni; Andrade, Danieli; Sakamoto, Ana Paula; Trindade, Vitor; Novak, Glaucia V.; Molinari, B.; Campos, Lúcia M A; Aikawa, Nádia E.; Pereira, Rosa Maria Rodrigues; Terreri, Maria Teresa; Magalhães, Cláudia Saad; Marini, Roberto; Gomes, H. R.; Silva, Marco F.; Oliveira, Sheila Knupp Feitosa de; Sztajnbok, Flávio; Sacchetti, Silvana; Bica, B.; Sena, Elisama Araújo de; Moraes, Ana Paula; Santos, Maria Carolina dos; Robazzi, Teresa Cristina Martins Vicente; Spelling, Paulo F; Scheibel, Iloite Maria; Cavalcanti, André; Naka, Erica Naomi; Guimarães, Luciano Junqueira; Santos, Flávia Patrícia Sena Teixeira; Sampaio, Magda Carneiro; Bonfá, Eloísa; Silva, Clóvis A.

doi:10.1016/j.autrev.2020.102693

Cited by 25 publications

(26 citation statements)

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“…The hallmark of cSLE is the wide spectrum of clinical and laboratory abnormalities, particularly with the production of multiple autoantibodies against histone, nonhistone, cytoplasm, and nuclear proteins, and a marked increase in proinflammatory cytokines [ 11 , 12 , 14 – 16 ]. The clinical cSLE presentation spectrum is very diverse, varying from acute, severe, life-threatening disease to chronic condition with an intermittent or continuous course, and is rarely associated with spontaneous remission without treatment [ 1 , 2 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

et al. 2021

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Childhood-onset systemic lupus erythematosus (cSLE) is a prototype of a multisystemic, inflammatory, heterogeneous autoimmune condition. This disease is characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality. Racial/ethnic background, socioeconomic status, cost of medications, difficulty accessing health care, and poor adherence seem to impact lupus outcomes and treatment response. In this article, the management of cSLE patients is updated. Regarding pathogenesis, a number of potential targets for drugs have been studied. However, most treatments in pediatric patients are off-label drugs with recommendations based on inadequately powered studies, therapeutic consensus guidelines, or case series. Management practices for cSLE patients include evaluations of disease activity and cumulative damage scores, routine non-live vaccinations, physical activity, and addressing mental health issues. Antimalarials and glucocorticoids are still the most common drugs used to treat cSLE, and hydroxychloroquine is recommended for nearly all cSLE patients. Disease-modifying antirheumatic drugs (DMARDs) should be standardized for each patient, based on disease flare and cSLE severity. Mycophenolate mofetil or intravenous cyclophosphamide is suggested as induction therapy for lupus nephritis classes III and IV. Calcineurin inhibitors (cyclosporine, tacrolimus, voclosporin) appear to be another good option for cSLE patients with lupus nephritis. Regarding B-cell-targeting biologic agents, rituximab may be used for refractory lupus nephritis patients in combination with another DMARD, and belimumab was recently approved by the US Food and Drug Administration for cSLE treatment in children aged > 5 years. New therapies targeting CD20, such as atacicept and telitacicept, seem to be promising drugs for SLE patients. Anti-interferon therapies (sifalimumab and anifrolumab) have shown beneficial results in phase II randomized control trials in adult SLE patients, as have some Janus kinase inhibitors, and these could be alternative treatments for pediatric patients with severe interferon-mediated inflammatory disease in the future. In addition, strict control of proteinuria and blood pressure is required in cSLE, especially with angiotensin-converting enzyme inhibitor and angiotensin receptor blocker use.

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Section: Introductionmentioning

confidence: 99%

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

et al. 2021

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“…In total, 2767 articles were retrieved from the databases according to the study strategy. After screening based on the inclusion and exclusion criteria, 17 cohort studies [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] were enrolled ultimately (Figure 1). There were a total of 7144 people participating in all these studies, including 1289 males (Table 1).…”

Section: Resultsmentioning

confidence: 99%

Was Antiphospholipid Syndrome a Risk Factor of Stroke? A Systemic Review and Meta-Analysis of Cohort Studies

Zhao

Zhou

Xu³

et al. 2021

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Antiphospholipid syndrome (APS) is characterized by thrombosis. This systemic review and meta-analysis was to verify the hypothesis that APS might increase the risk of stroke. Studies were identified after literature searching of PubMed, Embase, and Cochrane. Newcastle-Ottawa Quality Assessment Scale Cohort Studies (NOQAS-C) was used to assess the quality of studies. The pooled effect with 95% confidence interval (95% CI) was calculated by random-effect model. I -square ( I 2 ) was used to test heterogeneity. Funnel plot was used to evaluate publication bias. A total of 17 cohort studies with overall high quality were included. There was no publication bias. Pooled hazard ratio of stroke occurrence in APS patients was 1.76 (1.39-2.21) with low heterogenicity and stable result from sensitivity analysis. In the analysis of subgroups, pooled risk ratios of stroke occurrence in patients with only positive antibodies of APS diagnosis were 1.75 (0.99-3.09), which for the APS patients with other autoimmune diseases were 14.70 (7.56-28.56). APS might be a risk factor of stroke, especially in patients with other autoimmune diseases.

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“…Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thrombo-inflammatory condition characterized by venous and arterial thromboembolic events and, rarely, pregnancy morbidity in adolescents [1][2][3][4][5][6][7][8][9][10]. Despite its rarity in childhood and adolescence, it is one of the main non-congenital causes of symptomatic thromboembolism in the pediatric population [11].…”

Section: Introductionmentioning

confidence: 99%

“…Despite its rarity in childhood and adolescence, it is one of the main non-congenital causes of symptomatic thromboembolism in the pediatric population [11]. The laboratory hallmark of APS is the persistent (≥ 12 weeks) presence of elevated titers of pathogenic antiphospholipid antibodies (aPL), including lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-β2-glycoprotein I antibodies (anti-β2GPI) [1][2][3][4][5][6][7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

“…Pediatric APS occurs in neonates through to adolescents and can be classified as primary APS (PAPS), without any associated disease, or secondary APS (SAPS), when associated with another underlying illness [6], mainly autoimmune conditions such as childhood-onset systemic lupus erythematosus (cSLE) [8,[12][13][14]. A more aggressive APS subset is named pediatric catastrophic antiphospholipid syndrome (CAPS).…”

Section: Introductionmentioning

confidence: 99%

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Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects

et al. 2021

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Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical manifestations. APS in children and adolescents typically presents with large-vessel thrombosis, thrombotic microangiopathy, and, rarely, obstetric morbidity. Non-thrombotic clinical manifestations are frequently seen in pediatric APS and may be present even before the vascular thrombotic events occur. We review insights into the pathogenesis of APS and discuss potential targets for therapy. The identification of multiple immunologic abnormalities in patients with APS reveals molecular targets for current or future treatment. Management strategies, especially for APS in adolescents, require screening for additional prothrombotic risk factors and consideration of counseling regarding contraceptive strategies, lifestyle recommendations, treatment adherence, and mental health issues associated with this autoimmune thrombophilia. The main goal of therapy in pediatric APS is the prevention of thrombosis. The management of acute thrombosis events in children and adolescents is the same as for primary APS, which involves isolated occurrences, and secondary APS, which is seen in association with another autoimmune disease, e.g., systemic lupus erythematosus. A pediatric hematologist should be consulted so other differential thrombophilic conditions can be eliminated. Therapy includes unfractionated heparin or low-molecular-weight heparin followed by vitamin K antagonists. Treatment of catastrophic APS involves triple therapy (anticoagulation, intravenous corticosteroid pulse therapy, and plasma exchange) and may include intravenous immunoglobulin for children and adolescents with this condition. New drugs such as eculizumab and sirolimus seem to be promising drugs for APS.

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Childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome: A multicenter study with 1519 patients

Cited by 25 publications

References 33 publications

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

Was Antiphospholipid Syndrome a Risk Factor of Stroke? A Systemic Review and Meta-Analysis of Cohort Studies

Managing Antiphospholipid Syndrome in Children and Adolescents: Current and Future Prospects

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