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It is now widely accepted that antiphospholipid antibodies (aPL) have direct pathogenic effects and that B cells, notably through aPL production, play a key role in the development of antiphospholipid syndrome (APS). Recent findings strengthened the implication of B cells with the description of specific B cell phenotype abnormalities and inborn errors of immunity involving B cell signaling in APS patients. In addition, it has been shown in preclinical models that cross-reactivity between APS autoantigens and mimotopes expressed by human gut commensals can lead to B cell tolerance breakdown and are sufficient for APS development. However, B cell targeting therapies are surprisingly not as effective as expected in APS compared to other autoimmune diseases. Elucidation of the B cell tolerance breakdown mechanisms in APS patients may help to develop and guide the use of novel therapeutic agents that target B cells or specific immune pathway. Copyright © 2021. Published by Elsevier B.V.


Yannick Dieudonné, Aurélien Guffroy, Vincent Poindron, Pauline Soulas Sprauel, Thierry Martin, Anne-Sophie Korganow, Vincent Gies. B cells in primary antiphospholipid syndrome: Review and remaining challenges. Autoimmunity reviews. 2021 May;20(5):102798

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PMID: 33722752

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