B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis
Background: Systemic sclerosis (SSc), also known as scleroderma, is a complex, chronic autoimmune disease characterized by fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation. The treatment of SSc has historically focused on symptom management and slowing down dis...
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MDPI AG
2025-01-01
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| Series: | Sclerosis |
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| author | Gerhard Zugmaier Matthias Klinger Marion Subklewe Faraz Zaman Franco Locatelli |
| author_facet | Gerhard Zugmaier Matthias Klinger Marion Subklewe Faraz Zaman Franco Locatelli |
| author_sort | Gerhard Zugmaier |
| collection | DOAJ |
| description | Background: Systemic sclerosis (SSc), also known as scleroderma, is a complex, chronic autoimmune disease characterized by fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation. The treatment of SSc has historically focused on symptom management and slowing down disease progression through conventional immune-suppressive agents. New therapeutic approaches have been emerging due to advances in understanding of the disease mechanisms, particularly in the areas of fibrosis, vascular involvement, and immune dysregulation. Methods: In this review of the literature, we discuss the current stage of development of B-cell-depleting immune therapies in SSc. Results: B-cell depletion therapy has become an area of growing interest in the treatment of SSc due to the role played by B cells in the pathogenesis of the disease. There is increasing evidence that B cells contribute to disease progression through multiple mechanisms. B cells in SSc are implicated in autoantibody production, cytokine production, and fibroblast activation. B cells are responsible for producing autoantibodies, such as anti-topoisomerase I (Scl-70) and anti-centromere antibodies, which are hallmarks of SSc. B cells release pro-inflammatory cytokines (such as interleukin-6 [IL-6] and transforming growth factor β [TGF-β]), which promote fibrosis and inflammation, they also contribute to the activation of fibroblasts, the cells responsible for excessive collagen production and fibrosis, a key feature of SSc. Conclusions: In light of these findings, therapies that target B cells are being investigated for their potential to modify the disease course in SSc, particularly by reducing autoantibody production, inflammation, and fibrosis. |
| format | Article |
| id | doaj-art-cdc8b15379dc474c8f6e09f4da8e0888 |
| institution | OA Journals |
| issn | 2813-3064 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | MDPI AG |
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| series | Sclerosis |
| spelling | doaj-art-cdc8b15379dc474c8f6e09f4da8e08882025-08-20T02:31:13ZengMDPI AGSclerosis2813-30642025-01-0131510.3390/sclerosis3010005B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic SclerosisGerhard Zugmaier0Matthias Klinger1Marion Subklewe2Faraz Zaman3Franco Locatelli4Amgen Research (Munich) GmbH, 81477 Munich, GermanyAmgen Research (Munich) GmbH, 81477 Munich, GermanyDepartment of Medicine III & Gene Center, University Hospital Munich, Ludwig-Maximilians-Universität, 81377 Munich, GermanyAmgen Inc., Thousand Oaks, CA 91320, USAIRCCS Ospedale Pediatrico Bambino Gesù, Catholic University of the Sacred Heart, 00163 Rome, ItalyBackground: Systemic sclerosis (SSc), also known as scleroderma, is a complex, chronic autoimmune disease characterized by fibrosis of the skin and internal organs, vasculopathy, and immune system dysregulation. The treatment of SSc has historically focused on symptom management and slowing down disease progression through conventional immune-suppressive agents. New therapeutic approaches have been emerging due to advances in understanding of the disease mechanisms, particularly in the areas of fibrosis, vascular involvement, and immune dysregulation. Methods: In this review of the literature, we discuss the current stage of development of B-cell-depleting immune therapies in SSc. Results: B-cell depletion therapy has become an area of growing interest in the treatment of SSc due to the role played by B cells in the pathogenesis of the disease. There is increasing evidence that B cells contribute to disease progression through multiple mechanisms. B cells in SSc are implicated in autoantibody production, cytokine production, and fibroblast activation. B cells are responsible for producing autoantibodies, such as anti-topoisomerase I (Scl-70) and anti-centromere antibodies, which are hallmarks of SSc. B cells release pro-inflammatory cytokines (such as interleukin-6 [IL-6] and transforming growth factor β [TGF-β]), which promote fibrosis and inflammation, they also contribute to the activation of fibroblasts, the cells responsible for excessive collagen production and fibrosis, a key feature of SSc. Conclusions: In light of these findings, therapies that target B cells are being investigated for their potential to modify the disease course in SSc, particularly by reducing autoantibody production, inflammation, and fibrosis.https://www.mdpi.com/2813-3064/3/1/5systemic sclerosisautoimmunityblinatumomabB-cell depletion therapy |
| spellingShingle | Gerhard Zugmaier Matthias Klinger Marion Subklewe Faraz Zaman Franco Locatelli B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis Sclerosis systemic sclerosis autoimmunity blinatumomab B-cell depletion therapy |
| title | B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis |
| title_full | B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis |
| title_fullStr | B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis |
| title_full_unstemmed | B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis |
| title_short | B-Cell-Depleting Immune Therapies as Potential New Treatment Options for Systemic Sclerosis |
| title_sort | b cell depleting immune therapies as potential new treatment options for systemic sclerosis |
| topic | systemic sclerosis autoimmunity blinatumomab B-cell depletion therapy |
| url | https://www.mdpi.com/2813-3064/3/1/5 |
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