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抗磷脂综合征:一种具有新出现治疗机会的抗体介导疾病。

Antiphospholipid Syndrome: An Antibody-Mediated Disease With Emerging Therapeutic Opportunities.

作者信息

Newman Thalia G, Knight Jason S

机构信息

University of Michigan Medical School, Ann Arbor.

出版信息

Arthritis Rheumatol. 2025 May 19. doi: 10.1002/art.43258.

DOI:10.1002/art.43258
PMID:40386969
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12353912/
Abstract

Antiphospholipid syndrome (APS) is an autoimmune thromboinflammatory disease characterized by vascular thrombosis, pregnancy morbidity, and other manifestations driven by antiphospholipid autoantibodies. In this review, we present a clinical case that illustrates some diagnostic and therapeutic challenges in managing severe, relapsing APS manifestations, including microvascular injury and diffuse alveolar hemorrhage. We then discuss recent advances in our understanding of APS pathophysiology, emphasizing the critical role of anti-β-2 glycoprotein I antibodies in triggering cell-specific inflammatory and thrombotic pathways. Finally, we speculate that achieving definitive disease control will ultimately require strategies that eliminate or effectively neutralize these pathogenic antibodies.

摘要

抗磷脂综合征(APS)是一种自身免疫性血栓炎症性疾病,其特征为血管血栓形成、妊娠并发症以及由抗磷脂自身抗体引发的其他表现。在本综述中,我们展示了一个临床病例,该病例说明了在管理严重的、复发性APS表现(包括微血管损伤和弥漫性肺泡出血)时所面临的一些诊断和治疗挑战。然后,我们讨论了目前对APS病理生理学认识的最新进展,强调了抗β-2糖蛋白I抗体在触发细胞特异性炎症和血栓形成途径中的关键作用。最后,我们推测,要实现对疾病的彻底控制最终将需要采取消除或有效中和这些致病抗体的策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3141/12750120/7c4cfc1e1779/ART-77-1626-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3141/12750120/cc213e48785e/ART-77-1626-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3141/12750120/7c4cfc1e1779/ART-77-1626-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3141/12750120/cc213e48785e/ART-77-1626-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3141/12750120/7c4cfc1e1779/ART-77-1626-g001.jpg

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本文引用的文献

1
Interaction of antiphospholipid antibodies with endothelial cells in antiphospholipid syndrome.抗磷脂抗体在抗磷脂综合征中与内皮细胞的相互作用。
Front Immunol. 2024 Jul 9;15:1361519. doi: 10.3389/fimmu.2024.1361519. eCollection 2024.
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抗磷脂综合征患者的核苷酸酶活性降低和中性粒细胞-血小板聚集增加。
Blood. 2024 Mar 21;143(12):1193-1197. doi: 10.1182/blood.2023022097.
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Calprotectin Impairs Platelet Survival in Patients With Primary Antiphospholipid Syndrome.钙卫蛋白可损害原发性抗磷脂综合征患者的血小板存活。
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Role of Lipid Rafts on LRP8 Signaling Triggered by Anti-β2-GPI Antibodies in Endothelial Cells.脂筏在内皮细胞中抗β2糖蛋白I抗体触发的LRP8信号传导中的作用
Biomedicines. 2023 Nov 24;11(12):3135. doi: 10.3390/biomedicines11123135.
6
The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria.2023年美国风湿病学会/欧洲抗风湿病联盟抗磷脂综合征分类标准。
Arthritis Rheumatol. 2023 Oct;75(10):1687-1702. doi: 10.1002/art.42624. Epub 2023 Aug 28.
7
Structural analyses of β-glycoprotein I: is there a circular conformation?β-糖蛋白 I 的结构分析:是否存在环形构象?
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8
Interaction of the antiphospholipid syndrome autoantigen beta-2 glycoprotein I with DNA and neutrophil extracellular traps.抗磷脂综合征自身抗原β-2 糖蛋白 I 与 DNA 和中性粒细胞胞外陷阱的相互作用。
Clin Immunol. 2023 Oct;255:109714. doi: 10.1016/j.clim.2023.109714. Epub 2023 Jul 30.
9
Activation of Platelet mTORC2/Akt Pathway by Anti-β2GP1 Antibody Promotes Thrombosis in Antiphospholipid Syndrome.抗β2糖蛋白 1 抗体激活血小板 mTORC2/Akt 通路促进抗磷脂综合征血栓形成。
Arterioscler Thromb Vasc Biol. 2023 Oct;43(10):1818-1832. doi: 10.1161/ATVBAHA.123.318978. Epub 2023 Jun 29.
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Arthritis Rheumatol. 2023 Aug;75(8):1407-1414. doi: 10.1002/art.42489. Epub 2023 May 18.