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      Management of Pediatric Systemic Lupus Erythematosus: Focus on Belimumab

      1 , 2 , 1 , 2 , 3

      Drug Design, Development and Therapy

      Dove

      belimumab, pediatric SLE, lupus treatment

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          Abstract

          Belimumab (Benlysta ®) is a fully humanized monoclonal antibody that inhibits B lymphocyte stimulator (BLyS, also known as B cell-activating factor of the tumor necrosis factor family) and was approved by the US Food and Drug Administration (FDA) and the European Medicines Evaluation Agency for the treatment of autoantibody-positive systemic lupus erythematosus (SLE) in adults with moderate disease activity. Belimumab was recently FDA approved for use in children with SLE between 5 and 17 years of age. This review discusses the key findings of the belimumab phase III trials in adult SLE (via intravenous and subcutaneous administrations), phase II trial in pediatric SLE (intravenous administration), and post hoc analyses. It also evaluates the current clinical trials of belimumab in specific SLE disease states and highlights the safety profile of belimumab. It discusses the clinical post-marketing use of belimumab in adults and children with SLE and concludes with our recommendations for the use of belimumab to treat pediatric SLE, including a look to the future with increased real-world use in children with SLE.

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          Most cited references 39

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          Systemic lupus erythematosus.

          Systemic lupus erythematosus is an autoimmune connective-tissue disorder with a wide range of clinical features, which predominantly affects women, especially from certain ethnic groups. Diagnosis is based on clinical assessment supported by investigations, including the finding of autoantibodies. Treatments range from antimalarial agents to corticosteroids and immunosuppressive agents. This Seminar draws attention to advances in the epidemiology, genetics, cardiovascular risks, lupus nephritis, CNS disease, the antiphospholipid syndrome, assessment of disease activity and damage, and pregnancy related and quality of life issues. New therapeutic approaches, such as biological agents and mycophenolate mofetil, will also be discussed.
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            The BAFF/APRIL system in SLE pathogenesis.

            Systemic lupus erythematosus (SLE) is characterized by multisystem immune-mediated injury in the setting of autoimmunity to nuclear antigens. The clinical heterogeneity of SLE, the absence of universally agreed clinical trial end points, and the paucity of validated therapeutic targets have, historically, contributed to a lack of novel treatments for SLE. However, in 2011, a therapeutic monoclonal antibody that neutralizes the cytokine TNF ligand superfamily member 13B (also known as B-cell-activating factor of the TNF family [BAFF]), belimumab, became the first targeted therapy for SLE to have efficacy in a randomized clinical trial. Because of its specificity, the efficacy of belimumab provides an opportunity to increase understanding of SLE pathophysiology. Although belimumab depletes B cells, this effect is not as powerful as that of other B-cell-directed therapies that have not been proven efficacious in randomized clinical trials. In this article, therefore, we review results suggesting that neutralizing BAFF can have effects on the immune system other than depletion of B cells. We also identify aspects of the BAFF system for which data in relation to SLE are still missing, and we suggest studies to investigate the pathogenesis of SLE and ways to refine anti-BAFF therapies. The role of a related cytokine, TNF ligand superfamily member 13 (also known as a proliferation-inducing ligand [APRIL]) in SLE is much less well understood, and hence this review focuses on BAFF.
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              The pathogenesis of systemic lupus erythematosus-an update.

              Systemic lupus erythematosus (SLE, lupus) is characterized by a global loss of self-tolerance with activation of autoreactive T and B cells leading to production of pathogenic autoantibodies and tissue injury. Innate immune mechanisms are necessary for the aberrant adaptive immune responses in SLE. Recent advances in basic and clinical biology have shed new light on disease mechanisms in lupus, with this review discussing the recent studies that offer valuable insights into disease-specific therapeutic targets. Copyright © 2012 Elsevier Ltd. All rights reserved.
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                Author and article information

                Journal
                Drug Des Devel Ther
                Drug Des Devel Ther
                DDDT
                dddt
                Drug Design, Development and Therapy
                Dove
                1177-8881
                25 June 2020
                2020
                : 14
                : 2503-2513
                Affiliations
                [1 ]Division of Pediatric Rheumatology, Steven and Alexandra Cohen Children’s Medical Center of New York , Lake Success, NY, USA
                [2 ]Department of Pediatrics, Zucker School of Medicine at Hofstra/Northwell , Hempstead, NY, USA
                [3 ]Center for Autoimmune, Musculoskeletal, and Hematopoietic Diseases Research, Feinstein Institute for Medical Research , Manhasset, NY, USA
                Author notes
                Correspondence: Joyce S Hui-Yuen Division of Pediatric Rheumatology, Steven and Alexandra Cohen Children’s Medical Center , 1991 Marcus Avenue, Suite M100, New Hyde Park, NY11040Tel +1 516 472 3700Fax +1 516 472 3752 Email jhuiyuen@nshs.edu
                Article
                216193
                10.2147/DDDT.S216193
                7323799
                © 2020 Guzman and Hui-Yuen.

                This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms ( https://www.dovepress.com/terms.php).

                Page count
                Tables: 2, References: 64, Pages: 11
                Categories
                Review

                Pharmacology & Pharmaceutical medicine

                lupus treatment, pediatric sle, belimumab

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